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PATHPROS > WBC DISORDERS - 1 > Flashcards

WBC DISORDERS - 1 Flashcards

Acute leukemias, Myeloproliferative neoplasms, Myelodysplastic syndrome

1
Q

Why do you get plethora in PV?

Deborah Dalmeida MD

A

Increased RBC mass causes distension of venous side of circulation

Deborah Dalmeida MD

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2
Q

What is this called?

Known case of CML now presenting with increasing anemia and thrombocytopenia, increased resistance to therapy, falling platelet counts, and increasing numbers of basophils and blasts.

Deborah Dalmeida MD

A

Accelerated phase of CML

Deborah Dalmeida MD

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3
Q

Thrombotic manifestations of Polycythemia Vera

Deborah Dalmeida MD

A

MI

DVT

Stroke

Hepatic veins- Budd Chiari Syndrome

Mesenteric and portal- Bowel infarction

Deborah Dalmeida MD

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4
Q

What is this phase called?

Known case of CML , now presenting with severe anemia, thrombocytopenia, peripheral blood smear showing numerous blast cells not containing Auer rods

Deborah Dalmeida MD

A

blast crisis of CML

Deborah Dalmeida MD

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5
Q
  1. What risk of transformation do all the Myeloproliferative neoplasms carry?
  2. List all the Myeloproliferative neoplasms

Deborah Dalmeida MD

A
  1. Acute myeloid leukemia(AML)
  2. a.CML
    b. PV
    c. ET
    d. Primary myelofibrosis

Deborah Dalmeida MD

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6
Q

What’s the special name for the derivative chromosme 22 wherein there is Reciprocoal translocation between chromosomes 9 and 22 producing an ABR-BCL fusion gene ?

Deborah Dalmeida MD

A

Philadelphia chromosome

Deborah Dalmeida MD

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7
Q
  1. Relative/ absolute polycythemia?

normal plasma volume

increased RBC mass

normal SaO2

Increased EPO

  1. List 2 conditions where this scenario may be encountered

Deborah Dalmeida MD

A
  1. Absolute- inappropriate
  2. Renal cell carcinoma/ Hepatocellular carcinoma

Deborah Dalmeida MD

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8
Q

elderly

weakness

prior history of radiation therapy

anemia+thrombocytopenia

ringed sideroblasts on bone marrow

image finding (see attached)

Deborah Dalmeida MD

A

Myelodysplastic Syndrome

Deborah Dalmeida MD

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9
Q

Look at clues provided and the image- diagnosis?

elderly

persistently elevated platelet count>450,000/microliter

JAK 2 mutation

Deborah Dalmeida MD

A

Essential Thrombocytosis

Deborah Dalmeida MD

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10
Q

Underlying mutation in Polycythemia Vera

Deborah Dalmeida MD

A

JAK 2

Deborah Dalmeida MD

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11
Q
  1. Erythropoietin levels in Polycythemia Vera - High/Low?
  2. Why?

Deborah Dalmeida MD

A
  1. Low
  2. Mutation of JAK 2 causes the transformed progenitor cells to have decreased requirement for EPO.

Deborah Dalmeida MD

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12
Q

Underlying mutation in primary myelofibrosis

Deborah Dalmeida MD

A

JAK 2 -50-60%

MPL - 5-10%

Deborah Dalmeida MD

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13
Q
  1. Known case of Polycythemia Vera, now presenting with splenomegaly.

What findings do you expect on BM biopsy?

  1. What is this phase of Polycythemia Vera called?

Deborah Dalmeida MD

A
  1. Fibrosis which obliterates the bone marrow. As a result of fibrosis, there is extensive extramedullary hematopoiesis leading to splenomegaly
  2. Spent phase

Deborah Dalmeida MD

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14
Q

Pan T cell lineage markers

Deborah Dalmeida MD

A

CD 3, CD 5

Deborah Dalmeida MD

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15
Q

minimum percentage of blasts in the bone marrow to term it a leukemia

Deborah Dalmeida MD

A

20%

Deborah Dalmeida MD

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16
Q

1.Diagnosis?

fever, sore throat, gray white membrane on tonsils, posterior auricular lymphadenopathy

Monospot test positive

2. Causative organism?

3. Peripheral smear finding?

Deborah Dalmeida MD

A
  1. Infectious Mononucleosis
  2. Epstein Barr Virus
  3. Lymphocytosis

Deborah Dalmeida MD

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17
Q

What is the cause for the appearance of the splenectomy specimen seen in this image?

Deborah Dalmeida MD

A

Extramedullary hematopoiesis

It expands the red pulp of the spleen, effaces the white pulp producing a ‘beefy red’ appearance.

Deborah Dalmeida MD

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18
Q

What do the inclusions seen in the cytoplasm of this cell represent?

Deborah Dalmeida MD

A

needle like azurophilic granules - Auer rods

Deborah Dalmeida MD

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19
Q

10 year old

thymic mass

PS: tumor cells larger than small lymphocyte, fine, delicate to stippled chromatin, inconpicuous nucleoli, scant agranular basophilic cytoplasm

CD2, CD 3, CD5, CD7, Tdt positive

Deborah Dalmeida MD

A

T- ALL

Deborah Dalmeida MD

20
Q

The test you do to distinguish between Leukemoid reaction and chronic myeloid leukemia?

Deborah Dalmeida MD

A

Leukocyte alkaline phosphatase level (LAP score)

Deborah Dalmeida MD

21
Q

Identify the condition

Deborah Dalmeida MD

A

CML

Deborah Dalmeida MD

22
Q

List 4 risk factors for AML

Deborah Dalmeida MD

A

radiotherapy and chemotherapy

Fanconi anemia, Bloom syndrome

Benzene

preexisting myeloproliferative neoplasm or a myelodysplastic syndrome

Deborah Dalmeida MD

23
Q

Pan B cell lineage markers

Deborah Dalmeida MD

A

CD 10, 19, 20

Deborah Dalmeida MD

24
Q

Lymphoblast/ myeloblast?

tumor population composed of cells exhibiting delicate nuclear chromatin, two to four nucleoli, and more voluminous cytoplasm , containing fine, peroxidase-positive azurophilic granules.

Deborah Dalmeida MD

A

Myeloblast

Deborah Dalmeida MD

25
Q

elderly

dragging sensation in left upper quadrant

WBC>100,000/mm3

Neutrophils, myelocytes, metamyelocytes, band forms

eosinophilia, basophilia

thrombocytosis

hypercellular marrow

Deborah Dalmeida MD

A

CML(Chronic myeloid leukemia)

Deborah Dalmeida MD

26
Q

1. Diagnosis?

45 year old weakness and fatigue

splenomegaly

WBC 75,000cells/mm3

BM 40% blasts

CD 33, CD 13 and CD 117 positive

2. Special histochemical stain?

Deborah Dalmeida MD

A
  1. AML
  2. MPO(myeloperoxidase) , Sudan Black

Deborah Dalmeida MD

27
Q

most common acute leukemia of adults

Deborah Dalmeida MD

A

AML

Deborah Dalmeida MD

28
Q

Translocation in CML

Deborah Dalmeida MD

A

t(9;22)

Deborah Dalmeida MD

29
Q

Which subtype of AML is associated with a reciprocal translocation between chromosomes 15 and 17 producing a PML-RAR alpha fusion gene?

Deborah Dalmeida MD

A

Acute promyelocytoc leukemia (APML)

Deborah Dalmeida MD

30
Q

3 year old child

fatigue,fever

splenomegaly

WBC count 100,000 cell/mm3

anemia, thrombocytopenia

image findings (attached image)

CD 19, CD 20 and Tdt positive

Deborah Dalmeida MD

A

B cell- ALL

Deborah Dalmeida MD

31
Q

1. Diagnosis?

Very high leukocyte count

schistocytes, teardrop cells on blood smear

nucleated RBCs on blood smear

2. List 2 conditions where this reaction is seen

Deborah Dalmeida MD

A
  1. Leukoerythroblastic reaction
  2. a. Myelopthisis- bone marrow infiltration by tumor, granulomas etc
    b. Primary Myelofibrosis

Deborah Dalmeida MD

32
Q

Most common hematologic malignancy of children

Deborah Dalmeida MD

A

Acute lymphoblastic leukemia/ lymphoblastic lymphoma

Deborah Dalmeida MD

33
Q

Diagnosis?

Hb 14-28mg/dl

Hematocrit>60%

low EPO

Hypercellular marrow

PS- erythrocytosis, granulocytosis, thrombocytosis, basophilia

Deborah Dalmeida MD

A

Polycythemia vera

Deborah Dalmeida MD

34
Q

1. Diagnosis?

Elderly

progressive anemia, massive splenomegaly

PS- teardrop cells, nucleated RBCs

Markedly elevated WBC count

  1. Special stain?

Deborah Dalmeida MD

A
  1. Primary myelofibrosis
  2. Reticulin , Masson’s trichrome

Deborah Dalmeida MD

35
Q

most common cause for agranulocytosis

Deborah Dalmeida MD

A

Drug toxicity

  • alkylating agents
  • antimetabolites

Deborah Dalmeida MD

36
Q
  1. One important complication of APML?
  2. Translocation assoc with APML?
  3. Treatment?

Deborah Dalmeida MD

A
  1. DIC
  2. t(15;17)
  3. All trans retinoic acid

Deborah Dalmeida MD

37
Q
  1. Absolute/ relative polycythemia?

decreased plasma volume

normal RBC mass

normal SaO2

Normal EPO

  1. List 2 conditions where this finding may be encountered

Deborah Dalmeida MD

A
  1. Relative polycythemia
  2. Dehydration, diuretic use

Deborah Dalmeida MD

38
Q

What is the consequence of formation of the BCR-ABL fusion gene?

Deborah Dalmeida MD

A

constitutive activation of the ABL kinase

Deborah Dalmeida MD

39
Q
  1. Relative/ absolute polycythemia?

normal plasma volume

increased rbc mass

decreased SaO2

increased EPO

  1. List 2 conditions where this may be encountered

Deborah Dalmeida MD

A
  1. Absolute polycythemia - appropriate type
  2. chronic bronchitis, cyanotic congenital heart disease, high altitude

Deborah Dalmeida MD

40
Q

LAP score in Leukemoid reaction versus LAP score in CML

Deborah Dalmeida MD

A

Leukemoid reaction- elevated LAP

CML- depressed/ low in CML

Deborah Dalmeida MD

41
Q

1. Diagnosis?

Extreme elevation of leukocyte count (>50,000/μL) composed of mature and/or immature neutrophils.

No blasts

History of a serious infection

Deborah Dalmeida MD

A

Leukemoid reaction

Deborah Dalmeida MD

42
Q

Underlying mutation in CML

Deborah Dalmeida MD

A

BCR-ABL fusion gene

Deborah Dalmeida MD

43
Q

One complication of Infectious mononucleosis

Deborah Dalmeida MD

A

Splenic rupture

Deborah Dalmeida MD

44
Q

Why do you get acquagenic pruritus in Polycythemia vera?

Deborah Dalmeida MD

A

Histamine release

Deborah Dalmeida MD

45
Q

lymphoblast/ myeloblast?

cell larger than small lymphocyte, fine, delicate to stippled chromatin, inconpicuous nucleoli, scant agranular basophilic cytoplasm

Deborah Dalmeida MD

A

Lymphoblast

Deborah Dalmeida MD

46
Q

Mechanism of erythromelalgia (burning sensation of hands and feet) in Polycythemia vera?

Deborah Dalmeida MD

A

Microvascular occlusion and platelet activation

Deborah Dalmeida MD

PATHPROS (36 decks)

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