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HEME/LYMPH > WBC Disorders VIII > Flashcards

WBC Disorders VIII Flashcards

1
Q

chimeric BCR-ABL gene

A

chronic myeloid leukemia - CML

MPD

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2
Q

reciprocal (9;22) (q34;q11) translocation

A

philadelphia chromosome

90% creates the BCR-ABL gene of CML

BCR - 22
ABL - 9

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3
Q

myeloproliferative disorders

A

mostly - multipotent progenitor that gives rise to erythrocytes, platelets, granulocytes

less common - pleuripotent giving rise to lymphoid and myeloid clls

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4
Q

CML clinical

A

adult 50yo
males

fatigue, weak, weight loss, anorexia

abdominal pain/fullness - dragging sensation in abdomen
-due to EMH

LUQ pain - splenic infarct

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5
Q

sea blue histiocytes

A

scattered macrophage with wrinkled green-blue cytoplasm

in CML

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6
Q

diagnosis of CML

A

leukocytosis - possibly >100,000
hypercellular bone marrow - granulocytes, eosinos, basos, megas
-sea blue histiocytes and reticulin

LOW LAP**

detection of Ph1 or BCR-ABL fusion gene - chromosomal analysis or PCR based tests

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7
Q

LAP

A

leukocyte alkaline phosphatase

  • low in CML
  • high in reactive node - leukamoid rxn
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8
Q

CML prognosis

A

untreated - slow progression

3 year survival - no tx

50% accelerated phase - blast crisis in 6-12 months
-to AML (majority) or ALL

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9
Q

ikaros mutation

A

lymphoid blast crisis in CML

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10
Q

Tx of CML

A

BCR-ABL inhibitor - remission 90%

young pt - HSC transplant

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11
Q

panmyelosis

A

increased platelets

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12
Q

polycythemia vera

A

increased red cells, granulocytes, and platelets

RBC increase - polycythemia - most of symptoms

low EPO levels

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13
Q

low EPO, JAK 2 mutation, bone marrow study

A

polycythemia vera

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14
Q

P. rubra vera

A

polycythemia vera

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15
Q

PCV

A

polycythemia vera

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16
Q

clinical for polycythemia vera

A

late middle age
-insidious onset

pruritis, HA, HTN, GI ulcers, splenomegaly

elevated hematocrit**

bleeding and thrombosis

thrombocytosis >500,000 and many abnormal

DVT, MI, stroke

hyperuricemia - gout

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17
Q

Tx of polycythemia vera

A

phlebotomy - control RBC mass
-increase life expectancy from months to 10 years

JAK2 inhibitors

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18
Q

spent stage

A

polycythemia vera

aftet 10 years

fibrosis of marrow - and massive splenomegaly (due to EMH)

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19
Q

no tx with PCV

A

die from bleeding in months

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20
Q

essential thrombocytosis

A

increased platelet count
no polycythemia

point mutation in JAK2 or MPL

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21
Q

JAK2 or MPL mutation

A

essential thrombocytosis

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22
Q

essential thrombocytosis clinical

A

uncommon
adults > 60yo
insidious onset

high peripheral platelet count

platelets enlarged and abnormal

dx of exclusion - rule out other MPDs

23
Q

dx of essential thrombocytosis

A

bone marrow biopsy

-megakaryocyte significant increase

24
Q

tx of essential thrombocytosis

A

chemotherapy

25
Q

essential thrombocytosis prognosis

A

survival 12-15 yrs

progression to AML uncommon

26
Q

ET

A

essential thrombocytosis

27
Q

erythromelagia

A

throbbing and burning hands and feet because of small arteriole occlusion and platelet aggregation

with ET

28
Q

primary myelofibrosis

A

obliterative marrow fibrosis caused by non-neoplastic fibroblasts

difficult to treat

pt >60yo

29
Q

PDGF and TGF-beta

A

fibroblast activators

increased in primary myelofibrosis

30
Q

JAK2 and MPL mutations

A

present in primary myelofibrosis

31
Q

agnogenic myeloid metaplasia

A

early in progression of primary myelofibrosis

32
Q

normocytic, normochromic anemia with leukoerythroblastosis on peripheral smear

A

primary myelofibrosis

33
Q

primary myelofibrosis prognosis

A

death 3-5 years

-complications from cytopenias

34
Q

tx primary myelofibrosis

A

JAK2 inhibitor

HSC transplant in young

35
Q

tear drop shaped RBCs

A

dacrocytes
-damaged from fibrotic marrow

seen in primary myelofibrosis

36
Q

histiocytoses

A

macrophages and dendritic cells

37
Q

S100, CD1a, HLA-DR

A

positive in langerhans cell histiocytosis

38
Q

langerhans cell histiocytosis

A

considered neoplastic

39
Q

birbeck granules

A

racket shaped
-vesicular nuclei with linear grooves or folds and vacuolated cytoplasm

in langerhans cell histiocytosis

40
Q

mutations in BRAF

A

langerhans cell histiocytosis and hairy cell leukemia

41
Q

letterer-siwe disease

A

multifocal/multisystem langerhans cell histiocytosis

42
Q

seborrheic eruption

A

skin lesion in multifocal LCH

43
Q

LCH

A

langerhans cell histiocytosis

44
Q

multifocal/multisystem LCH clinical

A

before age 2

cutaneous lesions, fever, infection, chronic otitis media, mastoiditis

hepatosplenomegaly, lymphadenopathy, pulmonary and bone lesions

45
Q

multifocal/multisystem LCH prognosis

A

rapidly fatal

intense chemo - 50% 5 year survival

46
Q

eosinophilic granuloma

A

unifocal and multifocal/unisystem LCH

-langerhans cells mixed with eosinos, lymphocytes, plasma cell, neutrophils

47
Q

unifocal LCH

A

skeletal system in older children

calvarium, ribs, femur

48
Q

multifocal unisystem LCH

A

erosive bony masses in children

diabetes insipidus - pituitary stalk of hypothalamus

49
Q

diabetes insipidus

A

multifocal unisystem LCH

50
Q

hand schuller christain triad

A

calvarial bone defect
diabetes insipidus
exopthalmos

in multifocal unisystem LCH

51
Q

tx for multifocal unisytem LCH

A

chemotherapy

52
Q

tx for unifocal LCH

A

local excision - or radiation - cures most

53
Q

adult smoker

A

pulmonary LCH

54
Q

pulmonary LCH

A

multiple fine nodules and cysts in middle upper long zones

-BRAF mutation - neoplastic

with cigarette smoking**

cessation - may regress**

rare

HEME/LYMPH (11 decks)

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