Myeloid Neoplasms discreet facts Flashcards

1
Q

Chemotherapeutic agent that induces DNA mutations that can be related to development of hematopioetic neoplasms

A

Alkylating agents

also Topoisomerase Inhibitors (much faster rate of development after chemo)

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2
Q

Type of receptor that underlies genetic disorders of myeloproliferative neoplasms?

A

Tyrosine Kinase

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3
Q

Tyrosine kinase inhibitors treat Chronic Myeloid Leukemias however patients whose CML have developed a mutation within the ________ will not respond to the TKIs though 2nd and 3rd generation TKIs overcome these mutations?

A

in the ATP binding pocket that prevents drug from binding and also

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4
Q

Accelerated phase of CML can result in blast crisis which is a sudden and catastrophic ability to mature. The 2 types of Blast crisis is

A

70% Acute Myeloid Leukemia

30% is Acute Lymphoid Leukemia

This indicates that CML comes from pluripotential stem cell

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5
Q

Point mutation in pseudokinase domain of ______ involved in Polycythemia Vera

A

JAK2

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6
Q

JAK2 inhibitor that is pretty toxic

A

Ruxonitib

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7
Q

Presentation for early stages of Polycythemia Vera

A

Increased RBC mass: plethora, headache, dizziness, angina, visual disturbances, intermitted claudication (pain in walking)

normochromic, normocytic RBCs until later in the disease

low or normal serum EPO

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8
Q

What lab value would help to distinguish Polycythemia vera from a reactive polycythemia?

A

low or normal serum EPO or JAK2 mutation

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9
Q

Treatment of Polycythemia

Vera

A

Phlebotomy

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10
Q

Thrombopoietin receptor mutated in Essential Thrombocytosis or Primary Myeloproliferative disorder

A

JAK2

Calreticulin

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11
Q

Tear drop shaped RBCs

A

RBCs that have passed through areas of fibrosis in the bone marrow

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12
Q

Extramedullary hematopoeisis leading to _________ from Primary Myelofibrosis

A

Splenomegaly

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13
Q

How do you distinguish between reactive thrombocytosis or essential thrombocytosis (neoplastic)

A

Find a clonal abnormality to dx essential thrombocytosis

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14
Q

Mastocytosis mutation

A

c-Kit

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15
Q

Localized cutaneous manifestation of Mastocytosis?

A

Urticaria Pigmentosa

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16
Q

which myeloproliferative neoplasm has erythroid hyperplasia

A

polycythemia vera

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17
Q

which myeloproliferative neoplasm has Jak2 mutation?

A

Polycythemia vera
Primary myelofibrosis
Essential Thrombocytosis

18
Q

which myeloproliferative neoplasm has BCR/ABL fusion

A

Chronic Myeloid Leukemia

19
Q

c-Kit mutation

A

Mastocytosis

20
Q

Markedly increased platelets

A

Essential thrombocytosis

21
Q

Key morphology of acute leukemia

A

more than 20% myeloid cells in the bone marrow

22
Q

Drug association with Acute myeloid leukemia

A

Alkylating agents (very long lag time)

Topoisomerase inhibitors (short lag time)

23
Q

Trisomy assoc with Acute Myeloid Leukemia

A

Trisomy 21

24
Q

Acute Myeloid Leukemia is not one disease because it is the conglomeration of a bunch of genetic aberrations. It presents with

A

Anemia
Thrombocytopenia
Hepatosplenomegaly

25
Q

Flow cytometry used in AML pathology for

A

blast cell identification also cell surface markers

26
Q

Important genetics of AML

A

t 15;17

t 8;21

27
Q

Acute myeloid leukemia
t(15;17) disrupts retinoic acid receptor (RAR-alpha) gene

frequent association with _____ (histologically)

A

Auer Rods

28
Q

Treatment of acute promyelocytic leukemia

A

Trans retinoic acid to overcome the disrupted retinoic acid receptor very first (DO THIS WHILE WAITING ON CONFIRMATION)

29
Q

Diagnosis of AML genetics

A

PCR (takes a while because of long prep)
FISH (fastest test)
also morphology

30
Q

Morphology of AML

A

Auer Rods

Butterfly nucleus

31
Q

Dangerous Complication of Acute Myelogenous LEukemia

A

DIC

32
Q

Myelodysplastic Syndromes willl smolder and 10-40% turn into

A

Acute Myeloid Leukemia

33
Q

Myelodysplastic syndrome presents with what sort of neutrophils?

A

hypolobar neutrophils

34
Q

Langerhans Cell Histiocytosis show mutation in the ______ gene 60% of the time

A

BRAF

35
Q

Indolent disease usually assoc with Langerhans Cell Histiocytosis in 5-30 yo

A

Eosinophilic Granuloma

36
Q

Disease in kids associated with Langerhans Cell Histiocystosis

A

Hand-Schuller-Christian Disease

37
Q

t15;17

A

AML (promyelocytic variant)

38
Q

BRAF mutation

A

Langerhans Cell Histiocytosis

39
Q

Monosomy 7

A

myelodysplasia, if seen in AML then bad prognosis

40
Q

histologically significant finding in Langerhans cells histiocystosis?

A

Birbeck Granules

41
Q

5q- is seen in

A

myelodysplasia, AML w/ bad prognosis

42
Q

inv(16) prognosis?

A

Favorable prognosis