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Flashcards in Myeloid Neoplasms discreet facts Deck (42)
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1

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

Alkylating agents

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

2

Type of receptor that underlies genetic disorders of myeloproliferative neoplasms?

Tyrosine Kinase

3

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?

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

4

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

70% Acute Myeloid Leukemia

30% is Acute Lymphoid Leukemia

This indicates that CML comes from pluripotential stem cell

5

Point mutation in pseudokinase domain of ______ involved in Polycythemia Vera

JAK2

6

JAK2 inhibitor that is pretty toxic

Ruxonitib

7

Presentation for early stages of Polycythemia Vera

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

8

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

low or normal serum EPO or JAK2 mutation

9

Treatment of Polycythemia
Vera

Phlebotomy

10

Thrombopoietin receptor mutated in Essential Thrombocytosis or Primary Myeloproliferative disorder

JAK2
Calreticulin

11

Tear drop shaped RBCs

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

12

Extramedullary hematopoeisis leading to _________ from Primary Myelofibrosis

Splenomegaly

13

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

Find a clonal abnormality to dx essential thrombocytosis

14

Mastocytosis mutation

c-Kit

15

Localized cutaneous manifestation of Mastocytosis?

Urticaria Pigmentosa

16

which myeloproliferative neoplasm has erythroid hyperplasia

polycythemia vera

17

which myeloproliferative neoplasm has Jak2 mutation?

Polycythemia vera
Primary myelofibrosis
Essential Thrombocytosis

18

which myeloproliferative neoplasm has BCR/ABL fusion

Chronic Myeloid Leukemia

19

c-Kit mutation

Mastocytosis

20

Markedly increased platelets

Essential thrombocytosis

21

Key morphology of acute leukemia

more than 20% myeloid cells in the bone marrow

22

Drug association with Acute myeloid leukemia

Alkylating agents (very long lag time)

Topoisomerase inhibitors (short lag time)

23

Trisomy assoc with Acute Myeloid Leukemia

Trisomy 21

24

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

Anemia
Thrombocytopenia
Hepatosplenomegaly

25

Flow cytometry used in AML pathology for

blast cell identification also cell surface markers

26

Important genetics of AML

t 15;17
t 8;21

27

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

frequent association with _____ (histologically)

Auer Rods

28

Treatment of acute promyelocytic leukemia

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

29

Diagnosis of AML genetics

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

30

Morphology of AML

Auer Rods
Butterfly nucleus