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34 Cards in this Set
- Front
- Back
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features of B-ALL
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young child with anemia, marrow growth and CNS involvement
tdt+,CD19+,CD10+ sheetlike growth poor prognosis if under 2, present in teens or adulthood, high blast count or philadelphia chrom. |
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features of T-ALL
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mediastinal mass in an adolescent boy with difficulty breathing
tdt+, CD3+, CD10+, CD2,4,5,7,8 lymphadenopathy and spleenomegaly sheets of immature blasts |
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features of CLL/small lymphocytic lymphoma
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males over 50
diffuse effacement of nodal arch. small lymphocytes with hypercondensed chromatin SMUDGE CELLS CD5+, CD20+, CD23+, CD19+ poor prognosis with ZAP70+, |
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features of follicular lymphoma
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painless lymphadenopathy
rare lymphocytosis paratrabecular BM diffuse spleen involvement cells with cleaved nuclei CD20, CD10, bcl6, BCL2 (14:18) usually transforms to DLBCL with p53 mutations and C-MYC treat with Rituximab and chemo |
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features of DLBCL
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older patients
rapid enlargement, rare blood involvement spleen isolated mass, tonsils and adenoids de novo or from follicular lymphoma lymphocytes 2x the size of normal |
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features of Burkitt's
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rapid mass with early spread
starry sky appearance MUST have C-MYC (8:14) CD10,CD20,bcl6, Ki-67 curable with short term chemo |
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feature of monoclonal gammopathy
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persistent M spike
homogeneous protein produced by clone of neoplastic Ig secreting cell, usually plasma cell |
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features of Waldenstrom's macroglobinemia
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caused by lymphoplasmacytic lymphoma
IgM paraprotein causes hyperviscosity visual impairment, stroke, Raynaud's defective hematopoiesis due to infiltration NO lytic lesions bleeding and hemolysis mixture of mature lymphocytes, plasmacytoid lymphocytes and plasma cells treatable but not curable |
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features of primary amyloidosis
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usually lambda light chain
can cause acquired factor X def. along with other problems |
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features of monoclonal gammopathy of uncertain significance
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3% prevalence over 70
no myeloma symptoms no lytic lesions IgG under 3.5 g under 10% BM plasmacytosis |
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feature of multiple myeloma
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plasma cell infiltration of BM, over 30%
lytic lesions and reduced normal Ig's Benze Jones proteinuria rouleaux formation of RBC's Russel/Dutcher bodies increased Ca,renal dysfx.,anemia and bone involvement beta 2 microglobulin is the most important predictor RANKL and IgH translocations treatable but not curable |
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features of mantle cell lymphoma
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adult males
GI tract, spleen, BM and blood involved homogenous small lymphocytes cyclin D1/IgH rearrangement, (11,14) cyclin D1+ incurable with chemo |
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features of marginal cell lymphoma
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arise in inflamed mucosal sites- stomach
early stages regress with Ab infiltrate of medium sized B cells (11:18) or (1:14) or trisomy of 3,12,18 |
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features of hairy cell leukemia
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middle aged, white males
splenomegaly and pancytopenia fried egg appearance in BM with fibrosis CD25,CD11,CD103 and TRAP responds to 2-CDA |
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features of unspecified peripheral T cell lymphoma
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very aggressive
nodal CD2,3,5 small, intermediate and large lymphoid cells |
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features of anaplastic large cell lymphoma
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HALLMARK cells look like horseshoes
T cell lineage CD30 ALK+ translocation is good, seen in younger patients |
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features of adult T cell leukemia/ ATL
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skin lesions, lymphadenopathy, lymphocytosis, hyperCa due to osteoclast activation
CLOVERLEAF NUCLEI associated with HTLV-1 infection |
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features of mycosis fungoides
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adults or elderly with a RASH
CD4,25 associated Sezary syndrome-erythroderma,lymphadenopathy,neoplastic T cells |
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features of classical Hodgkin's lymphoma
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predictable spread and specific tx.
node to spleen to BM to liver,lung from germinal center or B cells lack Ig expression EBV association Reed-Sternberg cells CD30,15 good prognosis |
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features of lymphocyte predominant Hodgkin's
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males under 35
axillary and cervical lymphadenopathy CD20,bcl6, popcorn cells no EBV association |
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what is thymic hyperplasia associated with
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myastenia gravis
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features of DiGeorge syndrome
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failure of 3rd and 4th pharyngeal pouch development
thymic HYPOplasia and defect in T cell immunity hypoparathyroidism heart and vessel defects presents with hypocalcemia,CHF,cyanosis or infection |
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features of thymoma
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adults over 40
benign neoplasm of thymic ep. cell may present with myasthenia gravis or red cell aplasia cured with resection |
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features of acute myeloid leukemia/AML
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myeloid blasts replace the BM
repressed hematopoiesis-anemia,infx. recurrent translocations is good fine chromatin, Auer rods CD34,D1117 promyelocytic subtype presents with DIC, has a RARA fusion and responds to retinoic acid or arsenic tx. AML1/ETO fusion protein has best prognosis therapy related AML has worst prognosis(etoposide,alkylation) |
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features of myelodysplastic syndromes
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anemia with monocytosis or neutrophilia without infection,
abnormal marrow and PBC dysplasia anemia with many RBC precursors older patients may be therapy related or idiopathic |
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feature of chronic myeloproliferative diseases
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increased blood and BM cellularity with normal maturation
hepatosplenomegaly may progress to AML or ALL all show alterations in TK which are targets for tx. with small molecule inhibitors CML Polycythemia Vera Essential Thrombocythemia Idiopathic Myelofibrosis |
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features of Chronic Myelogenous Leukemia/CML
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long chronic phase transforming to acute leukemia
ABDOMINAL FULLNESS due to extreme splenomegaly BM increase in myeloid precursors increased neutrophils chronic phase has Bcr/Abl fusion(9:22) and low leukocyte alkaline phosphatase accelerated phase has more basophils and more Phil. chr.'s blast phase is acute leukemia with more blasts treat with imitinib |
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features of essential thrombocythemia
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unexplained elevated platelets
vascular occlusion-ischemia,gangrene large megakaryocytes burning of hands and feet JAK2 mutation may progress to AML with cytotoxic tx. |
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features of polycythemia vera
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RBC mass increase with low erythropoietin levels
HTN,thrombosis,headache,dizzy both bleeding and thrombosis, usually present with thrombosis marrow fibrosis leading to extramedullary hematopoiesis and organomegaly JAK 2 mutation AML risk with cytotoxic tx. |
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features of idiopathic myelofibrosis
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TEARDROP cells
very large spleen and liver collagen deposition by fibroblasts |
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features of Langerhans histiocytosis
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BIRBECK granules (tennis rackets)
young kids with skull involvement origin from immature dendritic cell multiple erosive bony masses multisystem variant is Letterer-Siwe disease bone involvement with pituitary compression, bulging eyes and DI is Hand-Schuller-Christian syndrome |
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what is the triad of hypersplenism
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splenomegaly
sequestration of RBC,WBC or platelets correction by splenectomy |
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what causes red pulp expansion
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cirrhosis
splenic vein thrombosis heart failure |
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what causes white pulp expansion
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infection
inflammatory disease ITP |
