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162 Cards in this Set
- Front
- Back
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What makes up the CBC?
What is a CBC differential count? Which WBCs are found in descending order? |
CBC = RBC, WBC, PLT
Differential = types of leukocytes or WBCs Neutrophils, Lymphocytes, Monocytes, Eosinophils, Basophils "Never Let Monkeys Eat Bananas" |
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What is leukopenia?
What is neutropenia? |
Leukopenia = Decr. WBCs (PMNs, then lymphocytes)
Neutropenia = Decr. PMNs |
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If WBCs are found to be < 1000/ul and < 500/ul, then what will we observe?
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< 1000/ul = incr. susceptibility to infection
< 500/ul = incr. risk for severe & fatal infection |
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What are three types of leukopenias?
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1. Chronic benign neutropenia
2. Agranulocytosis = absence of PMNs 3. Lymphopenia = decr. lymphocytes |
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What three WBCs are called granulocytes?
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Neutrophils
Eosinophils Basophils "NEB" |
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What causes each of the three neutropenias?
* Chronic Benign Neutropenia * Agranulocytosis * Lymphopenia |
Chronic Bengin Neutropenia = asymptomatic
Agranulocytosis = depletion of marginating PMNs and bone marrow reserve Lymphopenia = congenital and acquired immunodeficiency syndromes AND corticosteroids |
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What are two causes of neutropenia?
What are some clinical examples of each? |
1. Inadequate or ineffective granulopoiesis
++ aplastic anemia or leukemias - cancer & radiation therapy - drug suppression - ethanol - viral infections 2. Accelerated removal or destruction of neutrophils - immune-mediated injury (drugs or idiopathic) - incr. peripheral utilization secondary to severe infections - splenic sequestration |
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What are two major morphologic changes seen in bone marrow biopsy in patients with leukopenias?
What are the causes of each? |
1. Hypercellularity
- excessive destruction of PMNs - ineffective granulopoiesis (megaloblastic anemia) 2. Hypocellularity - marked decr. granulocytes or decr. of all blood cell precursors |
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What are the sign and symptoms of leukopenia?
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SYMPTOMS
- malaise, chills, and fever (decr. WBCs) - marked weakness, fatigability (decr. RBCs) SIGNS - infections - agranulocytic angina = ulcerating, necrotizing lesions of: ** gingiva, floor of mouth, buccal mucosa, pharynx |
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What are three options for treating patients with leukopenia?
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1. removal of offending drug
2. antibiotics to control infections 3. hematopoietic GF (G-CSF) |
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What is leukocytosis?
What generally causes this to happen? |
Leukocytosis = incr. WBCs due to:
* leukemoid reactions (mimic leukemia) * infectious mononucleosis |
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What is infectious mononucleosis in layman's terms?
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IM is a disease caused by EBV, a member of the herpes virus family, common in college students.
The infection is usually characterized by: * fever, sore throat, inflammation of the lymph nodes - leading to a response from CTL (CD8+) cells and antibodies against EBV It usually resolves in 4-6 weeks but fatigue may linger |
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Classically a patient with IM presents with which 3 symptoms?
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1. fever
2. sore throat 3. lymphadenitis |
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What would a CBC + differential show in IM?
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1. Absolute lymphocytosis (WBC# 12,000-18,000) with 60% of WBCs are lymphocytes
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What would PBS show in IM?
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Large, atypical lymphocyts bearing "T cell markers", abundant cytoplasm, large nucleus with fine chromatin
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What are the four common physical findings in IM?
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1. Little or no fever, malaise, fatigue, and lymphoadenopathy
2. Fever of unknown origin 3. Hepatitis 4. Febrile rash resembling rubella |
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What other diagnosis must be considered in an IM patient?
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1. CMV
as it may induce a similar syndrome and can only be DIFFERENTIATED by SEROLOGICAL METHODS 2. Hodgkins lymphoma Atypical lymphocytes resemble Reed-Sternberg cells -- need special tests to distinguish 3. Leukemia Need to apply 'LAP stain' to distinguish |
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How would one treat a patient with IM?
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Patients with acute EBV mononucleosis should be encouraged to rest as much as possible and to refrain from active physical activity for 3 weeks
- Surgery is necessary for spontaneous splenic rupture - Otherwise, closely monitor and treat |
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What fatal complications may occur with IM?
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1. Splenic rupture after minor trauma
2. Hepatic complications incl. jaundice, incr. enzyme levels, and failure |
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What fatal complication may occur in immunocompromised patients with IM?
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EBV -> human malignancies
lack of T cell immunity, reactivation of latent B cell infection, and ULTIMATELY B CELL LYMPHOMA |
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What does a positive MONOSPOT TEST indicate?
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Positive heterophil antibody reaction -> IM present
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What are four common characteristics of X-linked lymphoproliferative syndrome?
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1. X-linked
2. Inability to mount immune response against EBV 3. SH2D1A gene mutation 4. SH2D1A gene mutation = no activation of T and NK cells |
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What are two causes of reactive lymphadenitis?
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1. Infections and nonmicrobial inflammatory stimuli
2. Lymphadenopathy (tonsilitis) |
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What is acute nonspecific lymphadenitis?
What is its clinical course? |
It is confined to a group of nodes draining a focal infection
It is generalized in systemic bacterial or viral infection |
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What three patterns are seen in chronic nonspecific lymphadenitis?
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1. Follicular hyperplasia (B cells responding)
2. Paracortical lymphoid hyperplasia (T cells responding) 3. Sinus histiocytosis (histocytes responding) |
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What are three causes of follicular hyperplasia?
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1. rheumatoid arthritis
2. toxoplasmosis *** 3. early stages of HIV infection *** |
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What four findings favor a diagnosis of follicular hyperplasia versus follicular lymphoma?
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1. preservation of lymph node
2. marked variation in shape, size of lymphoid nodules 3. mixed population of lymphocytes 4. prominent phagocytic & mitotic activity in germinal centers |
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What are two causes of paracortical lymphoid hyperplasia?
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1. viral infections (IM) or after smallpox vaccination
2. drug induced immune reactions (phenytoin) |
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What histiopathologic pattern is seen in lymph nodes draining cancers?
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- Distention and prominence of lymphatic sinusoids
- Hypertrophy of lining endothelial cells - Infiltration w/ histiocytes |
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What is Cat-scratch disease and how does it present?
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- Self-limited lymphadenitis secondary to "Bartonella henselae"
- Caused by feline scratch Present in 90% < 18yo with lymphadenopathy, node enlargement 2 weeks after scratch |
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What is observed in a lymph node biopsy of a patient with cat-scratch disease?
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- sarcoid-like granulomas
- accumulation of PMNs => central necrosis in granuloma |
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What four factors are important in diagnosing a patient with Cat-scratch disease?
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1. History of exposure to cats or other injury
2. Clinical findings 3. Positive skin test to microbial antigen 4. Distinctive morphologic changes in lymph node |
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What are the three origins of neoplastic WBC disorders?
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1. Lymphoid neoplasms
2. Myeloid neoplasms 3. Histiocytic neoplasms |
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What are four subcategories of lymphoid neoplasms?
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1. Non-hodgkin lymphomas (NHLs)
2. Hodgkin lymphomas 3. Lymphocytic leukemias 4. Lymphoid tumors = lymphocytes arrested at particular stage of differentiation |
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What are three subcategories myeloid neoplasms?
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1. Acute myelogenous leukemias (AML)
- incr. progenitor non-lymph blood cells in BM = immature 2. Chronic myeloproliferative disorders (MPDs) - incr. non-lymph blood cells = mature 3. Myelodysplastic syndromes - decr. blood cell count (cytopenias) - INEFFECTIVE HEMATOPOIESIS IN BM |
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What are two subcategories hystiocytic neoplasms?
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1. Proliferative lesions of histiocytes
2. Langerhans cell histiocytoses |
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What is the difference in leukemias and lymphomas?
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Leukemias: arise in BM -> spill into circulating blood
Lymphomas: arise as tumor masses within lymph nodes |
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Describe plasma cell dyscrasias.
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- Arises as masses within bone
- Systemic symptoms secondary to inappropriate Ig production - Complete or partial monoclonal Ig polypeptides |
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What are two groups of lymphomas and how are they different?
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1. Hodgkin lymphoma (HL)
- presence of neoplastic Reed-Sternberg giant cells - non-neoplastic inflammatory cells + CURABLE CANCER, BETTER PROGNOSIS 2. Non-hodgkin lymphomas (NHL) + WORSE PROGNOSIS |
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Which cells are commonly found in NHL? Which are greater in abundance?
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B-cell origin (80% to 85%) -- better survival
T-cell origin (uncommon) -- worse survival NK cells (uncommon) |
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The origin of cells can be easily classified based on their CD markers.
What are the markers for: T cells B cells NK cells Immature T and BT cells Myeloid cells Pluripotent, lymphoid, and myeloid progenitor cells |
T cells = CD2-4, CD7-8
B cells = CD10, CD19-20, mIg NK cells = CD16, CD56 Immature T and B cells = Tdt Myeloid cells = CD13-15, CD64 Pluripotent, lymphoid, and myeloid progenitor cells = CD34 |
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List the differences between HL and NHL in terms of
* Clinical presentation * Treatment * Prognosis |
CLINICAL PRESENTATION
[ HL ] - presence of reed-Sternberg giant cells - non-neoplastic inflammatory cells [NHL] - Follicular lymphomas = mimics normal B cell nodular growth pattern + BETTER PROGNOSIS - Diffuse lymphomas = nodular pattern is lost, effaement of normal lymph node + WORSE PROGNOSIS - Lymphoid neoplasms = disrupt immune regulatory mechs. -> immunodeficiency, autoimmunity -> incr. infections - Inherited or acquired immunodeficiency = incr. risk of lymphoid neoplasms (EBV) TREATMENT HL = ABVD, MOPP NHL = CHOP (+rituximab) PROGNOSIS HL has a much better prognosis than NHL |
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What are the four characteristics used for REAL classification of NHL?
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1. morphology
2. cell of origin (B, T/NK, hodgkin lymphoma) 3. clinical features 4. genotype |
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List 8 types of lymphomas.
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1. Precursor B and T cell lymphoblastic leukemia/lymphoma
2. Small lymphocytic lymphoma/chronic lymphocytic leukemia 3. Follicular lymphoma 4. Mantel cell lymphoma 5. Diffuse large B-cell lymphomas 6. Burkitt lymphoma 7. Mutliple myeloma & plasma cell dyscrasias 8. Hodgkin lymphoma |
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What are two major pathophysiologic features of acute leukemias?
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1. Clonal proliferation & failure of maturation into mature cells => accumulation of neoplastic blasts
2. Suppression of normal hematopoietic stem cells in BM => leukemic blasts |
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What are the hematologic manifestation of acute leukemias?
What is their significance? |
Decr. RBCs -> anemia = normochromic, normocytic
Decr. WBCs -> infection = major cause of morbidity Decr. PLTs -> bleeding = BT abnromal |
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List two major differences between acute myeloblastic and lymphoblastic leukemia.
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1. Different response to therapy
2. Lymphoblasts have coarse, clumped chromatin and one or two nucleoli WHILE myeloblasts have finer chromatin and more cytoplasm containing granules |
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List 5 clinical features of acute leukemia.
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1. Abrupt stormy onset
2. Depression of normal BM function -> fatigue, fever, bleeding 3. Bone pain and tenderness 4. Lymphadenopathy, Splenomegaly, Hepatomegaly 5. CNS manifestations: headache, vomiting, nerve paslsies |
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What cells are increased or decreased in acute leukemias?
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Decr. RBCs, Hb, HCT
Decr. or Incr. WBCs (contrasting presentation) Incr. leukemic blast Decr. PLT# Pancytopenia (uncommon) Aleukemic leukemia (decr. blast in blood BUT incr. blast in BM = not able to get out) |
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What is immunophenotyping? How is it useful?
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- It's important in sub-typing lymphoblastic tumors
- Distinguishes ALL from AML - Tdt present in > 95% of ALL USEFUL IN PREDICTING CLINICAL OUTCOME |
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What is karyotyping? How is it useful?
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- It's an organized profile of a person's chromosomes
USEFUL IN PREDICTING PROGNOSIS |
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List 2 karyotypic changes in lymphoblastic leukemia/lymphoma associated with a good & poor prognosis.
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GOOD PROGNOSIS
- hyperdiploidy - t 12;21 POOR PROGNOSIS - pre-B-cell tumors = translocation ML1 gene on chromosome 11q23 - Philadelphia chromosome = t9;22 |
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What is the difference between SLL & CLL?
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Virtually identical tumors only differ in extent of peripheral blood involvement
CLL -> large # of circulating cells SLL -> none to little circulating cells |
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Who is most commonly affected?
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> 50 yo in western countries
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What would you expect to see on CBC + diff?
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Myeloid blasts (CD15, CD33, CD34)
B cells -> TdT, CD10, CD19, CD22 |
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What would you expect PBS to show?
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- Small, mature-looking lymphocytes
- Smudge cells (neoplastic lymphocytes are fragile & smudge) - Variable numbers of larger prolymphocytes |
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What would immunophenotyping show?
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- Neoplasm of mature (peripheral) B cells
- Pan-B-cell markers = CD19-20, CD23, sIgM and sIgD - EXPRESS T-CELL Ag = CD5 |
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List 4 sites of involvement.
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1. Lymph nodes
2. BM 3. Spleen 4. Liver |
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List 5 clinical features in SLL/CLL.
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1. Incr. susceptibility to bacterial infections
2. Autoimmune hemolytic anemia & autoimmune decr. PLTs 3. Lymphadenopathy & hepatosplenomegaly 4. Leukocytosis secondary to lymphocytosis in CLL 5. Slight leukocytosis in SLL |
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What's the clinical course & prognosis of SLL/CLL?
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- Live > 10 years after diagnosis & die of unrelated causes
- Median survival is 4 to 6 years |
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What pattern is seen on lymph node biopsy?
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Nodular aggregates of lymphoma cells in lymph node
-> small lymphoid cells with condensed chromatin & irregular or cleaved nuclear outlines |
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What would immunophenotyping show?
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- pan-B-cell-markers (CD19, CD20)
- restricted B-cell marker (CD10) - neoplastic cells express BCL2 protein |
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Who does follicular lymphoma more commonly affect?
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- Older persons, > 20 yo
- M=F |
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What is the clinical presentation?
What is the prognosis? |
- painless adenopathy & BM involvement
- translocation t(14;18) => overexpression of BCL2 PROGNOSIS - not easily curable - laock of response to chemotherapy due to anti-apoptotic effect - progression to diffuse B cell lymphoma (40% of cases) => assoc. with TP53 mutation PROGNOSIS IS LESS CURABLE FOR TRANSFORMED TUMORS THAN DE NOVO TUMORS |
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What role does BCL2 & TP53 gene play?
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BCL2 is antagonistic to the role of TP53 by blocking apoptosis
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What would a karyotype most likely show?
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translocation of t(14;18)
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Who does MCL most often affect?
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Affects older males
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What pattern of involvement would a lymph node biopsy show?
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Diffuse or vaguely nodular pattern
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What will PBS show at time of Dx of MCL?
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Irregular cleaved nucleus and inconspicuous nucleoli
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What will BM Bx show at presentation of MCL?
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B cell neoplasm resembling mantle zone cells
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List 5 clinical features of MCL
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1. fatigue & adenopathy
2. involvement of BM, spleen, liver, GI |
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What would immunophenotyping show?
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Coexpress mIgM, mIgD
CD19-21 ** CD5 ** |
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What would a karyotype show?
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t(11;14) -> dysregulated cyclin D1
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What is the clinical course & prognosis?
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aggressive, incurable
survival of 3-5yrs BAD PROGNOSIS |
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List 3 subtypes of DLBL
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1. Epstein-Barr virus (EBV)
2. Human herpesvirus type 8 (HHV-8) infection 3. Mediastinal large B-cell lymphoma |
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Who is affected by DLBL?
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- Older persons (median age of 60 yrs)
- 15% of childhood lymphomas |
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What is the typical clinical presentation?
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- rapidly enlarging, asymptomatic mass
- single nodal or extranodal site |
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What organs may become involved?
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GI tract, skin, bone, brain
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What will PBS most likely show?
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leukemic pictures rarely emerges
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What will immunophenotyping show?
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CD19-20, CD79a
IgM/IgG + kappa or lambda light chains CD10 variable expressed |
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What will karyotyping show?
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t(14;18)
BCL2 rearrangement BCL6 rearrangement |
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What is the clinical course & prognosis, if untreated?
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aggressive tumors, rapidly fatal if untreated
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What is the treatment & outcome?
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combination chemotherapy -> complete remission (60-80%)
50% disease free for several years -> considered cured |
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Where would patients with Burkitt’s lymphoma most likely reside?
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- Endemic in some parts of Africa
- Sporadic in other areas including US |
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What is the difference between the African & Non-African type?
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African: involvement of axilla or mandible
Non-african: abdominal tumors |
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What is the clinical course & prognosis?
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high-grade fastest growing human neoplasm
aggressive chemotherapy -> majority of patients cured |
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What other diagnosis must be considered if neoplastic lymphoblasts are seen on PBS?
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Must be distinguished from ALL
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What will immunophenotyping show?
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IgM
CD19 CD10 |
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What will karyotyping show?
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t(8;14)
translocation of MYC gene translocation fusing MYC +IgH gene |
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What role does the myc protein play?
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MYC protein has potent transforming activity
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List 6 major types of plasma cell dyscrasias?
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1) Multiple myeloma
2) Localized plasmacytoma (solitary myeloma) 3) Lymphoplasmacytic lymphoma 4) Heavy-chain disease 5) Primary or immunocyte-associated amyloidosis 6) monoclonal gammopathy of undetermined significance. |
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Which is the most common malignant plasma cell dyscrasia?
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Multiple myeloma (MM)
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What is Bence-Jones proteinuria?
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Finding kappa or lambda light chains in urine
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What is Light Chain Disease?
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It lacks a serum M component
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What is the M component and its importance?
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Homogeneous Ig identified in blood
- may be indicative of B cell malignancy - may also be seen in otherwise normal elderly persons |
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What is a localized plasmacytoma and what is its clinical presentation?
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Single lesion in skeleton or soft tissues
- Extraosseous lesions form tumorous masses in upper respiratory tract - Usually occult lesions elsehwere |
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What will blood test most likely show?
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Modest elevation in levels of M protein
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What is the clinical course & prognosis?
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May remain stale for several years but after 5-10 years
ost develop into multiple myelomas |
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What is the treatment for localized plasmacytoma?
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-
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What would you expect to see microscopic examination of a lymphoplasmacytic lymphoma?
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Mixed proliferation of B cells ranging from small round lymphs to plasmacytic lymphs to plasma cells
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What is the M component most likely composed of?
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monoclonal IgM
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What is Waldenstrom macroglobulemia?
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Hyperviscosity syndrome (large amount of IgM)
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What is the difference between IgG & IgA heavy chain disease?
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IgG: lymphadenopathy, hepatosplenomegaly
IgA: predilection for lymphoid tissues synthesizing IgA (GI & respiratory tract) |
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List 3 key features of Primary or Immunocyte-Associated Amyloidosis?
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1. Monoclonal proliferation of plasma cells
2. Excessive production of light chains 3. Amyloid deposits consist of partially degraded light chains |
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Who is most affected by Monoclonal Gammopathy of Undeterminded Significance (MGUS)?
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Asymptomatic, healthy persons > 50 yo
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What is the clinical course and prognosis?
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20% develop well-defined plasma cell dyscrasia in 10-15yrs
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List 2 things important in making the diagnosis of MGUS?
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< 3g /dL of monoclonal protein in serum
No Bence Jones proteinuria |
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Who is most affected by MM and how does it present?
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Persons between 50-60 yo
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List 7 features of myeloma nephrosis?
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1. Interstitial infiltrates
2. Proteinaceous casts 3. Multinucleate giant cells 4. Necrotic or atrophic cells lining tubules with casts 5. Metastatic calcification 6. Nodular glomerular lesions 7. Pyelonephritis |
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List 4 sites where neoplastic cells may be found in lymphoplasmacytic lymphoma
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1. BM
2. Lymph node 3. Spleen 4. Liver |
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List 2 major differences in the morphology & clinical course of lymphoplasmacytic lymphoma versus multiple myeloma
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MORPHOLOGY
CLINICAL COURSE |
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List 8 major clinical features of MM
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1. bone pain
2. pathologic fractures 3. hypercalcemia 4. Anemia 5. Recurrent infections 6. Hyperviscosity syndrome 7. Renal insufficiency 8. Amyloidosis |
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What will x-ray show in MM?
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X-ray shows punched-out defects in bone, usually 1-4cm in diameter
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What will serum on urine protein electrophoresis show?
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99%, monoclonal spike of Ig or light chains
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Who is most affected by lymphoplasmacytic lymphoma?
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Older person 50-60 yo
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Why does lymphoplasmacytic lymphoma result in a hyper-viscosity syndrome?
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Waldenstrom macroglublinemia -> large amount of IgM
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List 4 clinical features of Waldenstroms Macroglobuliemia (WM)
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1. Visual impairment
2. Neurologic problems 3. Bleeding 4. Cryoglobulinemia |
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What is the prognosis for MM and WM?
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MM: progressive disease, 2-4 yrs survival
LPL: 4-5 yrs survival |
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Explain the diagnosis of HL in laymen’s terms
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It is a malignant disorder involving lymphoid tissue (lymph nodes)
It can rise from a single node or chain of nodes Spreads anatomically to contiguous nodes |
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List 3 ways HL differs from NHL
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1. Reed-sternberg cells
2. Distinctive clinical features 3. Different treatment required |
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What are the four stages of HL?
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Stage 1- cervical, supraclavicular nodes
Stage 2 - above diaphragm Stage 3 - below diaphragm Stage 4 - bone marrow |
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What is a Reed-Sternberg (RS) cell and its significance?
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- Abundant, slightly eosinophilic, cytoplasm
- Multilobate nucleus or multiple nuclei - Large, round, prominent nucleoli TWO MIRROR-IMAGE NUCLEI OR NUCLEAR LOBES WITH LARGE ACIDOPHILIC NUCLEOLUS SURROUNDED BY A DISTINCTIVE CLEAR ZONE *** RS is a sign of HL |
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List 4 subtypes of HL
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1. Nodular sclerosis
2. Mixed Cellularity 3. Lymphocyte predominance 4. Lymphocyte depletion |
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List 2 subtypes in which “classic” RS are commonly found
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1. Nodular sclerosis
2. Mixed Cellularity |
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List 2 subtypes in which RS cell variants are commonly found
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1. Nodular sclerosis
2. Lymphocyte predominance |
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List 2 morphologic features that differentiates the types of HL
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* Morphology of neoplastic elements (RS cells)
* Number of RS cells relative to reactive lymphocytes |
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What is the most common HL subtype?
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Nodular sclerosis
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List 2 key morphologic features of Nodular Sclerosis HL
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* Lacunar cell = RS cell variant
* Collagen bands |
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What CD markers are expressed on RS & RS variant cells?
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CD15, CD30
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What is the most common type of HL in patients >50 y.o.?
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Mixed cellularity
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Which types of HL present with disseminated disease & systemic manifestations?
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Mixed cellularity
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What is the clinical presentation & prognosis of Lymphocyte Predominance HL?
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* Large # of small, mature-looking reactive LYMPHOCYTES admixed with variable number of BENIGN HISTIOCYTES
* L&H (lymphocyte & histiocyte) variant cells that have a POPCORN CELL (delicate, multilobed, puffy) |
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List 2 unique features of Lymphocyte Predominance HL
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* Typical nodular growth pattern
* L&H variants (popcorn cells) express CD20 *** EXCELLENT PROGNOSIS *** |
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Which HL subtype is found more commonly in women and usually affect teens & young adults?
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Nodular sclerosis
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List 4 common sites of involvement with progression of HL subtypes
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Spleen, liver, BM, lymph nodes
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List 3 physical exam findings found in HL
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1. Splenomegaly
2. Hepatomegaly 3. Lymph nodes swelling |
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What role does EBV play & in what HL subtypes?
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* Suspected etiologic agent for malignant transformation
* Infection in immunodeficient pts -> Burkitt's & B-cell lymphoma & HL mixed cellularity |
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List 3 cytokines secreted by RS cells and their function
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IL5 - attractant & GF for eosinophils
TFB-beta - fibrogenic factor IL-13 - stimulate RS cells via autocrine signaling |
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What role does CD30 ligand play in HL and where is it produced
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Produced by inflammatory cells with the intent to help RS cells grow and survive
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List one common PE findings at presentation
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Painless enlargement of lymph nodes
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What are the stages and prognosis for patients who present with localized & systemic disease respectively?
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Stages 1 & 2 - localized disease -- BETTER PROGNOSIS
Stage 3 & 4 - systemic complaints -- BAD PROGNOSIS |
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With HL, the common tumor seen in most cases is __________________
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B lymphocytes
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What is the common denominator among all subtypes of HL?
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RS cells and variants
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List 5 types of miscellaneous lymphoid tumors
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1. Extranodal (MALT) marginal zone lymphoma
2. Hairy cell leukemia 3. Mycosis Fungoides and Sezary syndrome 4. Adult T-cell leukemia/lymphoma 5. Peripheral T-cell lymphomas |
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What is a MALT lymphoma and where does it arise?
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* Low-grade B-cell tumors
* Arise most commonly in MALT (mucous tissue) incl. salivary glands, small and large bowel, lungs * Also arises in nonmucosal sites: orbit, breast |
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List 3 diagnosis associated with extra-nodal MALT lymphoma
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1. Sjogren syndrome
2. Hasimoto thyroiditis 3. H. pylori infection in stomach |
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What is the treatment for extra-nodal MALT?
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Local excision or radiotherapy
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What might cytogenetic analysis show?
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Two recurrent cytogenic abnormalities:
t(1;14) -- BLC10 and IgH genes t(11;18) -- MALT1 & IAP2 genes |
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Who does Hairy Cell Leukemia (HCL) affect?
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Older males
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List 2 factors responsible for the clinical manifestation of HCL
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* Leukemic cells with fine, hairlike cytoplasmic projections
* Express pan-B-cell markers CD19-20, mIg, CD11c/CD103 *** CD11c and CD103 USEFUL FOR DIAGNOSIS OF HAIRY CELL LEUKEMIA |
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What would you most likely find on PE?
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* Splenomegaly
* Pancytopenia |
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What would a CBC + diff show?
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Decreased WBCs
- pancytopenia - leukocytosis |
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What is the clinical course without treatment?
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Indolent but progressive disease if untreated
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What is the current treatment and prognosis for HCL
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Chemotherapeutic agents are workable
EXCELLENT PROGNOSIS |
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What is Mycosis Fungoides (MF) & Sezary syndrome?
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TUMORS OF PERIPHERAL CD4+ T CELLS
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How does MF present & progress?
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- cutaneous T cell lymphomas
- present as inflammatory premycotic phase -> progress through plaque phase -> tumor phase + Neoplastic T cells resembling popcorn cells |
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List 2 clinical manifestations of Sezary syndrome
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1. Exfoliative erythdroderma
2. Assoc. leukemia of sezary cells |
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Where would patient with HTLV-I infection most likely live or have traveled to?
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Southern Japan
Caribbean Southeastern USA (sporadic) |
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In addition to lymphoid malignancies, HTLV-I infection causes:
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1. Lymphoid malignancies
2. Progressive demyelinating CNS disease |
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List 6 characteristics of Adult T-cell Leukemia/Lymphoma
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1. Skin lesion
2. Hepatosplenomegaly 3. Hypercalcemia 4. Lymphocytosis w/ multilobed CD4+ lymphocytes 5. Lympadenopathy 6. Leukemic cells express high levels of IL-2 receptors |
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What is the clinical course and prognosis of Adult T-cell Leukemia/Lymphoma
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Extremely aggressive disease with medial survival time of ~8 months
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What is the presentation & prognosis of peripheral T-cell Lymphoma
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Presents as disseminated disease (stage IV)
Aggressive clinical course Responds POORLY to therapy |
