Hem & Onc - Pathology (Myeloproliferative disorders & related topics) Flashcards Preview

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Flashcards in Hem & Onc - Pathology (Myeloproliferative disorders & related topics) Deck (35):
1

What is Langerhans cell histiocytosis?

Proliferative disorders of dendritic (Langerhans) cells from monocytic lineage.

2

How does Langerhans cell histiocytosis present?

Presents in a child as lytic bone lesions and skin rash or as recurrent otitis media with a mass involving the mastoid bone.

3

Describe the functioning of cells in Langerhans cell histiocytosis.

Cells are functionally immature and do not efficiently stimulate primary T lymphocytes via antigen presentation.

4

What 2 markers are expressed by cells in Langerhans cells histiocytosis?

Cells express S-100 (mesodermal origin) and CD1a

5

What does the S-100 marker indicate, and with what myeloproliferative disorder is it associated?

S-100 (mesodermal origin); Langerhans cell histiocytosis

6

What EM finding is characteristic of Langerhans cell histiocytosis?

Birbeck granules ("tennis rackets" on EM) are characteristic

7

Name 4 chronic myeloproliferative disorders.

(1) Polycythemia vera (2) Essential thrombocytosis (3) Myelofibrosis (4) CML

8

What is important to remember about chronic myeloproliferative disorders in terms of their relation versus distinction?

The myeloproliferative disorders represent an often-overlapping spectrum, but the classic findings are described below.

9

With what process is JAK2 involved? How does it relate to myeloproliferative disorders?

JAK2 is involved in hematopoietic growth factor signaling. Mutations are implicated in myeloproliferative disorders other than CML.

10

What is the hematocrit in polycythemia vera?

Hematocrit > 55%

11

What kind of mutation is associated with polycythemia vera, and in what gene is it?

Somatic (non-hereditary) mutation in JAK2 gene

12

How does Polycythemia vera often present?

Often presents as intense itching after hot shower.

13

What is a rare but classic symptom of Polycythemia, and what causes it?

Rare but classic symptom is erythromelalgia (sever, burning pain and reddish or bluish coloration) due to episodic blood clots in vessels of the extremities

14

What causes secondary polycythemia?

Secondary polycythemia is via natural or artificial increase in EPO levels

15

To which other chronic myeloproliferative disorder is Essential thrombocytosis similar? What distinguishes it?

Similar to polycythemia vera, but specific for overproduction of abnormal platelets --> bleeding, thrombosis

16

What are the effects of Essential thrombocytosis?

overproduction of abnormal platelets --> bleeding, thrombosis

17

What is the histological finding associated with Essential thrombocytosis?

Bone marrow contains enlarged megaryocytes

18

What is myelofibrosis?

Fibrotic obliteration of bone marrow

19

What are histological findings of Myelofibrosis?

Teardrop RBCs and immature forms of the myeloid line; Think: "Bone marrow is CRYING because it's fibrosed"

20

What change is associated with CML, and what 2 effects does it have?

bcr-abl transformation leads to increased cell division and inhibition of apoptosis

21

What is the treatment for CML?

Treatment: imatinib (Gleevec)

22

Give the change/presence of the following in polycythemia vera: (1) RBCs (2) WBCs (3) Platelets (4) Philadelphia chromosome (5) JAK2 mutations.

(1) increased (2) increased (3) increased (4) negative (5) positive

23

Give the change/presence of the following in Essential thrombosis: (1) RBCs (2) WBCs (3) Platelets (4) Philadelphia chromosome (5) JAK2 mutations.

(1) no change (2) no change (3) increased (4) negative (5) positive (30-50%)

24

Give the change/presence of the following in Myelofibrosis: (1) RBCs (2) WBCs (3) Platelets (4) Philadelphia chromosome (5) JAK2 mutations.

(1) decreased (2) variable (3) variable (4) negative (5) positive (30-50%)

25

Give the change/presence of the following in CML: (1) RBCs (2) WBCs (3) Platelets (4) Philadelphia chromosome (5) JAK2 mutations.

(1) decreased (2) increased (3) increased (4) positive (5) negative

26

What are the 4 types of polycythemia?

(1) Relative (2) Appropriate absolute (3) Inappropriate absolute (4) Polycythemia vera

27

Give the change in the following with Relative polycythemia: (1) Plasma volume (2) RBC mass (3) O2 saturation (4) EPO levels.

(1) decreased (2) no change (3) no change (4) no change

28

Give the change in the following with Appropriate absolute polycythemia: (1) Plasma volume (2) RBC mass (3) O2 saturation (4) EPO levels.

(1) no change (2) increased (3) decreased (4) increased

29

Give the change in the following with Inappropriate absolute polycythemia: (1) Plasma volume (2) RBC mass (3) O2 saturation (4) EPO levels.

(1) no change (2) increased (3) no change (4) increased

30

Give the change in the following with polycythemia vera: (1) Plasma volume (2) RBC mass (3) O2 saturation (4) EPO levels.

(1) increased (2) very increased (3) no change (4) decreased

31

What causes relative polycythemia? Give 2 examples of this.

Decrease in plasma volume (dehydration, burns)

32

What are 3 examples of conditions associated with Appropriate absolute polycythemia?

(1) Lung disease (2) Congenital heart disease (3) High altitude

33

What are 5 examples of conditions associated with Inappropriate absolute polycythemia?

(1) Renal cell carcinoma (2) Wilms tumor (3) cyst (4) hepatocellular carcinoma (5) hydronephrosis

34

What causes inappropriate absolute polycythemia?

Due to ectopic EPO

35

What causes polycythemia vera?

Due to negative feedback