Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
218 Cards in this Set
- Front
- Back
what are the 3 characteristics of hematologic neoplasms?
|
1. lineage (myeloid vs. lymphoid), 2. survival (aggressiveness - acute vs. chronic), 3. predominant site of involvement (blood and marrow vs. tissues)
|
|
what type of cells are predominant in acute neoplasms?
|
immature cells - blasts or very early precursors
|
|
what type of cells are predominant in chronic neoplasms?
|
mature cells
|
|
what is the predominant site of involvement in leukemias?
|
blood and bone marrow
|
|
what is the predominant site of involvement in lymphomas?
|
lymphoid tissue
|
|
what is the predominant site of involvement in granulocytic sarcoma?
|
myeloid tissue
|
|
what is the predominant site of involvement in plasmacytoma or myeloma?
|
plasma cells - tissue
|
|
what are the 4 chronic myeloproliferative neoplasms?
|
1. chronic myeloid leukemia (CML), 2. PV, 3. essential thrombocytemia, 4. primary myelofibrosis
|
|
what are the characteristics of myelodysplastic syndromes?
|
cytopenias, dysplasia (abnormal cell appearance), impaired funciton; tendency to transform to acute leukemia
|
|
define NHL
|
neoplastic, clonal proliferation of lymphcytes; cells may retain properties of normal cells - produce Ig or cytokines; have systemic circulation or selective circulation
|
|
what is the demographics of NHL?
|
males, 60-70s, whites > blacks
|
|
what are the characteristics of myeloproliferative disorders?
|
clonal stem cell disorders which retain the capacity to differentiate, all lineages can be involed but a single one usually dominates in a pt
|
|
define myelodysplastic syndrome
|
stem cell disorders characterized by abnormal and inefficient maturation and differentaion
|
|
what are the predisposing factors of NHL?
|
chemicals - pesticides, fertilizers, 'agent orange'; ionizing radiation; viruses (HTLV-1, EBV, HCV); H.pylori; immunosuppression, autoimmune disease
|
|
what type of cytogenic alterations are found in NHL?
|
usualy reciprocal translocations - with proto-oncogenes and ither Ig genes or TCR genes
|
|
what is the most common locus involved in HNLs?
|
14q - Ig heavy chain gene
|
|
t(8;14)
|
NHL: Burkitt lymphoma - c-MYC/IgH
|
|
t(14;18)
|
NHL: follicular and diffuse large cell lymphoma - IgH/BCL-2
|
|
t(11;14)
|
NHL: mantle cell lymphoma - Cyclin D1/IgH
|
|
what is the function of 8q24?
|
MYC oncogene - drives cell proliferation
|
|
what is the function of 14q32
|
Ig heavy chain gene (IgH)
|
|
what is the function of 18q21?
|
bcl-2: prevents cell death due to apoptosis
|
|
what is the funciton of 11q?
|
gene for cyclin D1 - involvedin regulation of the cell cycle
|
|
what is the most common presentation of HNL?
|
lymphadenopathy
|
|
what hematologic diseases are associated with NHL?
|
lymphocytosis, leukopenia, anemia, thrombocytopenia
|
|
what are the advantages and disadvantes of WF classification
|
pro: simple, clincially useful; con: ignores phenotpe, cytogenetics and molecular tests
|
|
what are the 3 prognostic groups of WF classification?
|
1. low grade - survival > 5 years; 2. intermediate - survival 2-3 years; 3. high grade - survival<1.5 years
|
|
what are the histology characteristics that are used in the WHO classification?
|
architecture: follicular vs. diffuse and cell size: small vs. large
|
|
what type of NHL histology is more aggressive?
|
diffuse architecture with large cell size have a more aggressive clincial behavior
|
|
what is the % of HNL made up by histological grade?
|
50% made up of intermediate grade (diffuse large B-cell); 25% low grade (follicular lymphomas), true high grade lymphomas (burkitt and lymphoblastic lymphomas) are rare
|
|
what are the clinical characteristics of indolent NHL?
|
majority diseminated, predominantly nodal +/- liver, spleen and BM, long median survival, relentless relapse, incurable; can transform to high grade
|
|
what are the clinical characteristics of aggressive NHL?
|
may be localized at diagnosis, extranodal not common, short median survival if not treated, long disease free-survival possible, significant population curable
|
|
which indolent NHLs most often transform into aggressive ones?
|
follicular lymphomas and small lymphocytic lymphomas
|
|
what is the most common NHL?
|
diffuse large B-cell lymphoma - aggressive
|
|
what factors aid in prognosis of diffuse large B-cell lymphoma?
|
germinal center B-cell type - better prognosis, activated B-cell type - worse prognosis
|
|
what is the cure rate of diffuse large B-cell lymphoma?
|
40% with combo chemo
|
|
what is the second most common NHL?
|
follicular lymphoma
|
|
what is the most common indolent lymphoma?
|
follicular lymphoma
|
|
what is the histology like of follicular lymphomas?
|
the follicles are uniform in size and shape and uniformly distributed throughout the node; reactive - are usually different size and shape and not uniformily distributed
|
|
what are the grades of follicular lymphomas?
|
grade 1: fewest large cells; grade 2 and grade 3: most large cells (treated as aggressive)
|
|
what is the most common extranodal lymphoma?
|
MALT (marginal zone) lymphoma
|
|
where do marginal zone lymphomas occur?
|
50% in GI - mostly stomach
|
|
what is the behavior of marginal zone lymphomas?
|
indolent - POTENTIALLY CURABLE if they are localized
|
|
what type of cells make up marginal zone lymphomas
|
B-cells
|
|
what is a predisposing factor for gastric MALT lymphomas?
|
H. pylori - lymphoma goes away if H.pylori is eradicated
|
|
what is small lymphocytic lymphoma?
|
Idental to CLL - but lack lymphocytosis; low grade, long medial survival; 3-5 % evolve into large cell lymphoma
|
|
histology: complete effacement of normal lymph node architecture by a diffuse proliferal of small lymphocytes with some clusters of slightly larger cells
|
SLL
|
|
what is unique about mantle cell lymphomas
|
one of the worst lymphomas: short survival time and incurable
|
|
what are the demographics of mantle cell lymphomas?
|
older men; 5-10% of all lymphoma; advanced stage at diagnosis
|
|
what lymphoma has the fastest doubling time?
|
Burkitt lymphoma; very common HIV associated lymphoma
|
|
what is associated with Burkitt lymphoma?
|
EBV - in the african variant; presents with huge jaw mass +/- abdmominal disease
|
|
which lymphoma presents as an intra-abdominal disease?
|
Burkitt lymphoma - often ileo-cecal area (mimics appendicits)
|
|
which lymphoma has a starry sky histology with cattered large histiocytes with clear cytoplasms that are phagocytizing the neoplastic cells
|
burkitt lymphoma
|
|
what is the clinical behavior of T-cell lymphomas?
|
aggressive; EXCEPT - mycosis fungoides, a low grade cutaneous lymphoma with a long survival rate
|
|
what are the characteristics of T-cell lymphomas at diagnosis?
|
often disseminated, associated with systemic symptoms and extranodal involvement
|
|
what is the most common type of T-cell lymphoma?
|
peripheral T-cell lymphoma, unspecified (50%)
|
|
what Ann Arbor stage has single site involvement?
|
Stage 1
|
|
what Ann Arbor stage has more than one site but on the same side of the diaphragm?
|
stage 2
|
|
what Ann Arbor stage has diseae on both sides of the diaphragm?
|
Stage 3
|
|
what Ann Arbor stage has disseminated extranodal disease +/- nodal disease?
|
stage 4
|
|
what sites of involvement always indicate stage 4 on the Ann Arbor staging scale?
|
liver and bone marrow
|
|
what does "A" denote in the Ann Arbor staging system?
|
absence of systemic symptoms
|
|
what does "B" denote in the Ann Arbor staging system?
|
presence of systemic symtoms
|
|
what is very important in classifying T-cell lymphomas?
|
site of involvment is key
|
|
how are low grade NHL treated?
|
palliatively
|
|
how are high grade NHLs treated?
|
aggressively with combo chemo with intent to cure
|
|
what is a common regimen for treating high grade NHLs?
|
CHOP- often combined with Rituxan (anti-CD20 mab)
|
|
what are the 6 clinical prognostic factors for NHL?
|
histology,
age (older, worse), stage (localzied, better), LDH (elevated, worse), perfoamce status of pt, number of extranodal site |
|
what types of NHL are associated with HIV?
|
aggressive types - Burkitt, large cell immunoblastic, diffuse large b-cell lymphoma; lymphoma of the CNS is common
|
|
which type of cell is more commonly involved in lymphoma associated with HIV?
|
B-cell; NHL > HL; frequently with extranodal cites; EBV associated - MYC rearrangments
|
|
what is a predisposing factor for NHL associacated with HIV
|
increasing incidence with lower CD4 counts
|
|
what are the characteristics of NHL in transplant pts?
|
often high stage, extranodal sites, aggressive, B-cell> T-cell, EBV involved
|
|
what organ transplants are more associated with NHL?
|
heart and lung > heart or liver> kidney
|
|
which transplant associated NHL responds well to treatment?
|
polymorphic B-cell hyperplasia may resopnd to decreased immunosuppression
|
|
which transplant associated NHL does not respond well to treatment?
|
malignant lymphoma
|
|
when is a fine needle aspirate cytology a good option?
|
deep-seated or difficult to reach sites; that are non-lymphoid malignancies; it is non-invasion; can be combined withflow cytometry; NOT good for HL
|
|
what are the benefits of a excisional biopsy?
|
allows good histology with plenty of tissue, but it is invasive, must have OR, difficult for deep seated sites, and difficult if pt is really sick
|
|
how should you handle a lymph node biospy?
|
lymph node should be intact, given to pathology immediately, fresh- do not put in formalin or other fixatives
|
|
what characterizes HL?
|
RS cells (binucleated with chromatin clearing and alrge eosinophilic neucloi that look like owl's eyes) ina background of benign lymphcytes and other cells (eosinophils, plasma cells and histocytes)
|
|
HL vs. NHL: spread
|
HL- orderly, contiguous; NHL - disseminated, non-contiguous
|
|
HL vs. NHL: involvment site
|
HL - central and axial nodes; NHL - both central and peripheral nodes
|
|
HL vs. NHL: mesenteric nodes and Waldeye'rs ring involvment:
|
HL - seldom; NHL - often
|
|
HL vs. NHL: extranodal presentation?
|
HL- rare; NHL - not uncommon
|
|
what are the demographics of HL?
|
bimodal distribution - 20's (either sex) and >60s; incidence increases with educational level
|
|
what type of HL is most common in pts in their 20s?
|
nodular sclerosis
|
|
what type of HL is most common in pts >60?
|
mixed cellularity type - more aggressive
|
|
what are the predisposing factors to HL?
|
1. genetics, 2. EBV, 3. immunosuprression (May be a cause or may be a consequence)
|
|
what is the cell of origin for nodular lymphocyte predominant subtype of HL?
|
B- lymphocytes - may be cause of other HLs
|
|
what are the 4 types of HL in the Rye classification?
|
1. lymphocytic predmoniant, 2. nodular sclerosis, 3. mixed cellularity, 4. lyphocytic depletion
|
|
what is different about the WHO classification of HL vs. Rye?
|
who separaed nodular lymphocyte predmoniant HL from others which they call classical HL: nodular sclerosis, mixed cellularity, lymphocyte rich and lymphocyte deplete
|
|
what are the characteristics of nodular lymphocyte predmoninant HL?
|
Male, young, often localized - cervial, axillary or inguinal nodes; 5% of HLs
|
|
what is the course of nodular lymphocyte predominant HL?
|
indolent, long survival; may relapse late; may transform to large cell NHL
|
|
rare RS cells, predominance of background cells in a lymph node that is divided into nodules
|
nodular lymphocyte predominant HL
|
|
what is the HL where male = female?
|
nodular sclerosis
|
|
what location is commonly involved in nodular sclerosis HL?
|
mediastinal, cervial or supraclavicular (above the diaphargm) usually localized and stage II at diagnosis
|
|
bands of collagen dividing lymph node into nodules, with a fibrosis capsule, and lacunar RS cells
|
nodular sclerosis
|
|
what is the second most common HL?
|
mixed cellularity type - more aggressive
|
|
histology: more RS cells, fewer benign cells, background can have fibrosis
|
mixed cellularity type - more aggressive
|
|
histology: rare RS cells, predominantly lymphocytes and other benign cells
|
lymphocyte-rich HL
|
|
what is the presentation of lymphocyte rich HL?
|
older, men>women, low stage at diagnosis, indolent course, prolonged survival
|
|
what are the characteristics of lymphocyte-deplete HL?
|
rare - many that were caleed LDHL are now called NHL; older, males, advanced stage and aggressive course
|
|
what are the 2 variants of lymphocyte-deplete HL?
|
1. abundnat RS with paucity of benign cells and 2. diffuse fibrosis with rare RS cells
|
|
what does pathologic staging require?
|
staging laparotomy - biospy of multiple lymph node groups, splenectomy and multiple liver biospies; used to decide between radiation alone or with chome
|
|
what are the prognostic factors for HL?
|
worse if:
>45 yoa, bulky mediastinal diseaes, systemic symptoms, invovlement of bone marrow, liver, pleura and/or multiple extranodal sites; incrased LDH, beta2-microgolbulin and ESR |
|
when is radiation used in HL therapy?
|
as adjuvant for bulky disease
|
|
what are the 4 types of CLL?
|
B-cell chronic lymphocytic leukemia (CLL), hairy cell, prolymphocytic leukemias
|
|
what is the most common adult leukemia in the US?
|
CLL
|
|
what is the demographics of CLL?
|
older, men, more common in agricultural and asbestos works, some genetics
|
|
what is the most common cause of chronic lymphocytosis in audlts?
|
CLL
|
|
which neoplasms consists of monoclonal lymphyctosis of small, mature appearing B-cell
|
CLL
|
|
what are the 2 variants of CLL?
|
pre-germinal center: "unmutated IgH - more aggressive; and post-germinal center - mutate IgH - less aggressive
|
|
what is the presentation of CLL?
|
frequently asymptomatic - unexpected findings on CBC; lymphadenopahty common, can have hepatomegaly
|
|
how do you diagnose CLL?
|
lymphocytosis > 5000/uL, characteristic immunophenotype with small mature appearing lymphocytes and smudge cells
|
|
smudge cells are common in what?
|
CLL
|
|
what is the immunphenotype of CLL?
|
1. DIM surface monoclonal Ig expressed, b-cell markers and CD5
|
|
CD5 marker on B-cell lymphoma
|
CLL
|
|
what predicts aggressive CLL?
|
1. lymphocyte doubling time <12 months,
2. diffuse pattern of marrow infiltration, 3. presence of cytogenetic abnormaliteis, 4. bone marrow compromise, 5. unmutated IgH, 6. CD38 expression, 7. ZAP-70 expression |
|
what are the 2 staging systems used for CLL?
|
Rai and Binet - presence of bone marrow involvment puts you in high risk
|
|
what is the clinical course of CLL?
|
intermediate - 1 yr survival, 10% very indolent
|
|
what is the most frequent cause of death in CLL?
|
infection - most commonly respiratory tract (S. pneumonia, H. influenzae, S. aureas, pseudomonas, gram (+) enterococccie
|
|
what are the complications of CLL?
|
infection, anemia and thrombocytopenia, autoimmune phenomena - Abs agaisnt RBC, +DAT, mass effects, transformation into aggressive disease
|
|
what are infections in CLL due to?
|
hypogammaglobinulinemia
|
|
what is Richter's syndrome?
|
transformation of CLL to aggressive disease - 3-5% transform to large cell NHL
|
|
what is the DOC for CLL?
|
fludarabine - nucleoside analogue, can lead to opportunisitic infections
|
|
what are indications for treatment of CLL?
|
lymphocytosis is NOT an indication. Indications: 1. rapid lympohcyte doubling time, 2. bulky lymphadenoapthy, 3. bone marrow compromise, 4. autoimmune phenomena
|
|
what is hairy cell leukemia?
|
variant of CLL - uncommon; older adults 50s, men
|
|
what are the characteristics of hairy cell leukemia?
|
cytopenias, splenomegaly - often massive, distinictive "hairy" lymphocytes in blood
|
|
TRAP stain
|
hairy cell leukemia
|
|
bone marrow: lots of fibrosis with even spaced lymphoid cells surrounded by clear halo = fried egg cells
|
hairy cell leukemia
|
|
what is unusual about hairy cell leukemia and the spleen?
|
it involves the red pulp - most other involve the white pulp
|
|
splenomegaly - discomfort and early satiety and recurrent pyogenic infections
|
hairy cell leukemia
|
|
how do you treat hairy cell leukemia?
|
cladribine: nucleoside analogue, single dose causes durable complete resonses in many
|
|
lymphocytosis of T-cells with multilobated or convoluted nuclei
|
Adult T-cell leukemia/lymphoma
|
|
what neoplasm is associated with HTLV-1
|
Adult T-cell leukemia/lymphoma
|
|
what is the most common lymphoid maligancy in blacks?
|
multiple myeloma
|
|
plasma cells in bone marrow, bone lesions, monoclonal Ig or Ig fragments in serume or urine
|
multiple myeloma
|
|
what are the predisposing factors of multiple myeloma?
|
ionizaing radiation, increased in farms, petroleum, wood, leather and asbsetos industries
|
|
what is the most common cause of death in multiple myeloma?
|
infections - pneumonitis and pyelomehpritis are common
|
|
what is the cause of bone lesions in multiple myeloma?
|
osteoclast activiating factors produced by plasma cells - pathological fractures and bone pain is common
|
|
what are the 4 key features of multiple myeloma?
|
1. bone lesions, 2. infections, 3. renal insufficiency, 4. hypercalcemia
|
|
what is the second most common cause of death in multiple myeloma?
|
renal insufficiency
|
|
what is the diagnostic triad for multiple myeloma?
|
1. M-spike IgG > IgA (can be light chain only which won't have a spike),
2. monoclonal plasma cells in bone marrow and 3. end organ or tissue impariment: CRAB - calcium abnormalities, renal abnormalites, anemia and bone lesiosn |
|
what determines prognosis is multiple myeloma?
|
B2-micorglobulin - increases with increasing tumor burden; plasma cell labeling index increases with worse prognosis, anaplastic myeloma worse prognosis
|
|
what is the treatment for multiple myeloma?
|
hematopoeitic stem cells autologous or allogeneic if possible
|
|
monoclonal IgM with lymphoplasmocytic lymphoma
|
Waldenstrom's macroglobulinemia
|
|
what is the common feature of amyloidosis?
|
precipitation of protein in beta-pleated sheets
|
|
what is the most common type of amyloidosis?
|
AL type - Ig light chain fragments; Lambda > kappa
|
|
what is the second most common type of amyloidosis?
|
AA type: SAA protein - seen with chronic inflammatory conditions = RA
|
|
congo red apple-green birefringent
|
amlyoidosis
|
|
what organs are most often involved in amyloidosis?
|
heart, kidney, skin, liver, lungs, and nerves
|
|
when do you see AL type amyloidosis?
|
multiple myeloma or Waldenstrom's; but can be primary diseae
|
|
clonal proliferation of immature hematopoietic precursors with absent/impaired response to regulatory controls and imparied maturation
|
acute leukemias
|
|
what is suppressed in acute leukemias?
|
normal hematopoiesis - granulocytopenia, anemia, thrombocytopenia
|
|
what population is ALL common?
|
kids more than adults
|
|
what is the main cause of death in Acute leukemias?
|
suppression of normal hematopoiesis - infection and hemorrhage
|
|
what are the metabolic complications of acute leukemias?
|
hyperuricemia, hyperphosphatemia, tumor lysis syndrome with acute renal failure, hyper or hypo-kalemia
|
|
what is hyperviscocity syndrome?
|
medical emergency, occurs with high blast counts, AML >> ALL, CNS and respiratory symptoms; treat with leukapheresis and chemo
|
|
what type of cell is most common with ALL?
|
B cell precursors, the rest are precursor t-cells
|
|
basophilic cytoplasm and clear cytoplasmic vacuoles in mature B-cells
|
Burkitt cell leukemia
|
|
what are the prognosis factors for ALL?
|
age - 2-10 yoa best; WBC count - lower the better, phenotype early precursor B-cell - best; hyperdiploid - favorable, t(12;21) - favorable; t(9;22) or t(8;14) = both very unfavorable
|
|
how do you treat ALL?
|
3 phases: remission induction, consolidtaion (intensification) and maintianence - for 2-3 years
|
|
what is needed in treatment of ALL?
|
CNS prophylasxis - to prevent relapse
|
|
what is the most common leukemia in adults?
|
AML - increases with age male > female
|
|
t(8;21)
|
AML-M2 - diagnostic without blast count
|
|
t(15;17)
|
acute promyelocytic AML - diagnostic without blast count
|
|
inv(16)
|
AML-M4Eo - diagnostic without blast count
|
|
t(11q23)
|
Infant AML - M4, M5
|
|
deletion of 5, 7, 5q or 7a
|
therapy related AML
|
|
AML prognosis: t(8;21)
|
favorable
|
|
AML prognosis: t(15;17)
|
favorable
|
|
AML prognosis: Inv(16)
|
favorable
|
|
AML prognosis: t(11q23)
|
unfavorable
|
|
AML prognosis: deletions of 5, 7, 5q or 7q
|
unfavorable
|
|
myeloperoxidase stain
|
AML - diagnostic
|
|
auer rods
|
AML
|
|
what are the prognosis factors of AML?
|
better if: <40, de novo, low WBC count, DIC absent, LDH normal
|
|
what is unique about acute promyelocytic leukemia?
|
AML subtype that affects young pt, associated with DIC, and has a unique therapy: ATRA! T(15;17) PML/RARalpha
|
|
PML/RARalpha
|
acute promyelocytic AML
|
|
what is the major cause of death in myelodysplastic syndrome?
|
marrow failure (not leukemic transformation)
|
|
cytopenia, morphologic abnormalities in blood in marrow - with increased apoptosis and ineffective hematopoeisis
|
myelodysplastic syndrome
|
|
how do myelodysplastic syndromes present?
|
elderly men, nonspeciifc symptoms, recurrent infections or hemorrages
|
|
what neoplasm has decreased cells that are abnormal?
|
myelodysplastic syndrome
|
|
what is treatment-associated myelodysplastic syndrome?
|
occurs after chemo for HL, NHL; alkylating agents and nitrosoureas, usualy 5-10 yrs after therapy
|
|
ringed sideroblasts
|
myelodysplastic syndrome commonly causes these, can be seen with some meds, alcohol abuse and some inhertied conditions
|
|
what is 5- syndrome?
|
type of myelodysplastic syndrome - older pt, women, refracotry macrocytic anemia, normal or increased platelet count, 5q- is SOLE abnormality; prolonged survival
|
|
what are the prognosis factors for myelodysplasia?
|
older - worse, more cytopenias - worse, more blasts - worse, 7-, 7q-, 5- >3 abnormalities - worse
|
|
where are the msot common sites for relapse of ALL?
|
CNS, testes, marrow
|
|
chronic proliferation of hematopoietic stem cells with maturation and differentiation preserved leading to increased numbers of mature, relatively normal cells
|
chronic myeloproliferative neoplasms
|
|
which chronic myeloproliferative neoplasms have the greatest risk of transfomring to AML?
|
CML and primary myelofibrosis - PV or ET have a low risk
|
|
what are the cytogenic abnormaites found in chronic myeloprolifreative neoplasms?
|
TK - controls growth and STAT pathway; and JAK2-617F occurs in all non-CML variants
|
|
philadelphia chromosome
|
CML
|
|
t(9;22)
|
CML
|
|
bcr/abl
|
CML - codes for p210 that is a more active TK; rare cases make p190
|
|
what is a predisposing factor for CML?
|
ionizing radiation
|
|
histology: granulocytosis, all stages of maturation present, predmonient myelocytes and segs, increased basophils
|
CML
|
|
what is associated with increased basophils?
|
CML
|
|
what are the findings of CML?
|
splenomegaly - can be marked,
WBC high, thormbocytosis, LAP score low; GOUT can occur |
|
what is the course of CML?
|
chronic phase - 3or 4 years; blast crisis >20% blasts in marrow or blood; acclerated phase - increased blasts, WBC count - msot develop crisis; burn out - fibrotic phase resembling PM
|
|
what is the DOC for CML?
|
Gleevec, hydroxyuria can help with WBC count
|
|
what is Gleevec?
|
inhibitor of bcr/abl TK - blocks ATP binding site- initial TOC for chronic phase CML
|
|
what is the curative treatmetn for CML?
|
allogenic bone marrow transplant - the earlier the better
|
|
excess production of RBCs
|
polycythemia vera
|
|
what is the main risk of polycythemia vera?
|
thrombosis - treat with phlebotomy+/- hydroxyurea
|
|
marked increase in platelets
|
essential thrombocythemia
|
|
how can you distinguish PV from reactive process?
|
in PV EPO is low
|
|
hypercellular marrow with stainable iron
|
PV
|
|
pruritis after takinga hot shower
|
PV
|
|
"ruddy" skin - red/dark blotches
|
PV
|
|
what population does ET affect more?
|
women
|
|
giant platelets, bizarre shaped platetes, megakaryocyte nuclear fragments >600K platelet count
|
ET
|
|
clustering megakaryocytes in marrow
|
ET
|
|
leukoerythroblast reaction in blood, fibrosis in marrow, splenomegaly
|
PM
|
|
tear drop RBCs, nulceated RBCs, immature granulocyte precursors and giant platelets
|
PM
|
|
bone marrow stains for reticulin
|
PM
|
|
what are the major causes of death in PM?
|
hemorrhage, infection, heart failure, leukemic transformation
|
|
what kind of LAP score does CML have?
|
decreased
|
|
what is actually increased in PV?
|
RBC mass (not % that is erythrocytosis)
|
|
what leukemia can have thrombocytosis?
|
CML
|