03 Myeloid Leukemias and Reactive Disorders Flashcards

0
Q

Benign WBC disorders

A
Leukemoid reaction
Leukoerythroblastic reaction
Neutrophilia
Neutropenia
Eosinophilia
Basophilia
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0
Q

Leukoerythroblastic RXN

A

Immature BM cells in peripheral blood

Infiltration of BM- Mets, BM fibrosis
Stress- Sepsis or GF stress

i.e. Myeloblasts and nucleated RBCS

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0
Q

Neutrophilia

A

Neutrohils >7k

  • Infection
  • Sterile inflam and necrosis (MI)
  • Drugs- ie. lithium, steroids, catecholamines

Increased production or decreased margination

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1
Q

Leukemoid Rxn

A

50-80 or 90K Leukocytes
Neutrophils, lymphocytes, or eosinophils

Causes:

  • Perforating appendicitis(neut)
  • Bordatella (lymphocytes) - Whooping cough
  • Cutaneous larval migrans- Eosinophilia
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2
Q

Neutropenia

A

<1.5k Neuts

  • chemo
  • aplastic anemia
  • immune destruction (SLE)
  • Septic shock

Decreased production or increased margination/destruction

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2
Q

Eosinophilia

A

> .7k eosin

  • Asthma/allergy
  • parasitic
  • addisons/hypercortisol
  • Hodgkins lymphoma

Increased recruitment or production via leukotriene stimulation

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3
Q

Basophilia

A

> .2K

  • CML
  • CKD
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7
Q

MPNs

A

CML
Polycythema Vera
PMF (primary myelofibrosis)
Essential Thrombocythemia

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8
Q

Polycythema Vera

A

JAK mutation
Increased RBC mass, Leukocytosis, thrombocytosis
Decreased EPO

BM fibrosis and hypercellularity in late disease

Splenomegaly, thrombosis, gout, histamine

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8
Q

PMF

A
Primary myelofibrosis
TEARDROP RBCs
Leukoerythroblastic rxn - Immature cess in peripheral blood
Jak/MPL mutation
BM fibrosis and Megakaryocyte atypia
  • Splenomegaly with portal HTN,
  • Splenic infarct and reactive left pleural effusion
10 years in prefribrotic then 3-7 in fibrotic stages
5-30% get AML 
*BM failure
*Throbosis
*Portal HTN
*HF
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9
Q

CML t(9:22)

A

40-60 YO
Hepatosplenomegaly with weakness, weight loss, anorexia
WBC increased (100K): Mature Myeloid cells present with left shift
BM- Granulocytic hyperplasia
t(9:22) BCR-ABL fusion “philadelphia chromosome”
Chronic phase-3 years, accelerated 1 yr, then AML or ALL
Constitutive TK activity - treat with TKi - DASATINIB, IMATINIB (gleevec)

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10
Q

Myelodysplastic syndromes

A
Blasts <20% in BM and PB
Monosomy 5, 7  Trisomy 8
Leukoerythroblastic rxns and syplasia
hypercellular dysplasia
Hypolobulated, hypogranular,
Ring sideroblasts (rings or granules around RBC nuclei) - can be MDS drugs or alcohol
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10
Q

MDS Clinical

A

50-80 yo.
Weakness, infections, bleeding, or assymptomatic
9-29 month survival
30% progression to AML

Treatment:
Supportive
Hypomethylating agents - Decitabine, Azacitidine
SCT

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12
Q

Essential Thrombocythemia

A

Jak2 mutation
>450K atypical platelets
Hypercellular BM with abnormal megakayocytes
Giant hypogranular platelets

Bleeding, splenomegalu

12-15 year survival- treat with alkylating agents

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14
Q

0 AML

A
  • course of weeks to months
  • Adults age 60
  • 25% long term survival (poor prognosis overall)
  • large uniform blasts (>20% in BM or PB)
  • fine chromatin with many nucleoli
  • Cytoplams with granules
  • Auer rods!!!
  • myelodysplasia

Test with MPE or NSE enzyme assay (blue granules and bright red cytoplasm respectively)

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14
Q

AML clinical presentation

A
*Cytopenia- weakness, fatigue, petechiae, infections
Sometimes:
Orgaonmegaly
lymphadenopathy
extramedullary infiltration
rare coagulopathy
15
Q

AML with good prognosis

A

t(8:21)
Inv(16)
T(15:17)

16
Q

AML t(15:17)

A

Acute promyelocytic leukemia
hypergranular progmyelocytes with auer rods FAGGOT CELLS
Leukopenia with DIC
Treat with ATRA (screwed up RA signaling)
FAVORABLE PROGNOSIS

17
Q

AML with T(8:21)

A

Granulocytic AML with a few auer rods

FAVORABLE PROGNOSIS

19
Q

AML Inv(16)

A

Granulocytic and monocytic AML
Extramedullary involvemen
FAVORABLE PROGNOSIS

20
Q

AML 11q23

A

MLL
monocytic hyperleukocytosis
INTERMEDIATE TO POOR PROGNOSIS

21
Q

AML with MDS changes

A

Monosomy 5, 7

POOR PROGNOSIS

22
Q

ALL

A

Common in children(75% of childhood cancers) though 50% are adults
M>F
*small blasts
*High N/C
*Absent or scant nucleoli
85%b cell ALLs t(12;21), t(9;22)BCR/ABL (philadelphia chromosome has bad prog)
15% T ALL- adolescent males with mediastinal masses. Worse prognosis

24
Q

Langerhans cell histocytosis

A

CD1a langerin positive
*Tennis racket BIRBECK GRANULLES
BRAF MUTATIONS

25
Q

Hemophagocytic lymphohistocytosis

A

Primary: defects in perforin gene
Secondary: EBV and Lymphoma associated
*Hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis
FEVER