lleukemias/lymphomas 3

Question 0

Diagnostic marker for Hairy Cell Leukemia

Answer 0

TRAP (Tartrate Resistant Acid Phosphatase)

Question 1

Leukemia/Lymphoma associated with autoimmune hemolytic anemia

Answer 1

CLL (Chronic lymphocytic leukemia/lymphoma)

Proliferation of naive B cells that do not become plasma cells (hypogammaglobulinemia) (most common cause of death is infection)
When cells do try to become immune cells, they create antibodies that attack body’s own RBCs

Question 2

Why does Hairy Cell Leukemia not present with Lymphadenopathy?

Answer 2

Lymphadenopathy is enlargement of lymph nodes.
Because the mature B cells are trapped in the spleen (red pulp) and in the bone marrow (dry tap on aspiration), they do not go to the lymph nodes.

Question 3

Drug to treat Hairy Cell Leukemia

Answer 3

2-CDA

it’s a Adenosine Deaminase Inhibitor
accumulates to toxic levels in neoplastic B cells

Question 4

Adult T Cell Leukemia Lymphoma (ATLL)
is proliferation of?
Associated with what virus?

Answer 4

CD4+ T Cells

HTLV-1 (Human T Cell Leukemia Virus -1) (Japan/Caribbean)

Question 5

Symptoms of ATLL

Answer 5

Mature T cells like to go to skin so common symptom is Rash
(along with LAD and HSM)
***Lytic Bone Lesions (like Multiple Myeloma)

Question 6

What is Pautrier Microabscesses

Answer 6

Aggregates of neoplastic T cells in epidermis

Question 7

What is Sezary syndrome?

Answer 7

When mature T cells spread to blood

Characterized by lymphocytes with cerebriform nuclei on blood smear (looks like a brain)

Question 8

Why do you get hyperuricemia and gout in Myelogenous Leukemias?

Answer 8

Hyperproduction of cells results in increase in degradation as well. Increase in the Purine Degredation Pathway results in more uric acid, which leads to gout.

Question 9

Chronic Myelogenous Leukemia gene mutation

Answer 9

t(9;22) resulting in BCR-ABL fusion
increased tyrosine kinase activity -> cell growth

first line treatment: imatinib (blocks tyrosine kinase)

Question 10

CML can progress to…

Answer 10

ALL or AML

because mutation is in hemopoetic stem cells, which can develop into myeloid or leukoid cells

Question 11

How to tell difference between CML and leukemoid reaction?

Answer 11

CML granulocytes are LAP (leukocyte alkaline phosphatase) NEGATIVE. Because LAP is found in inflammatory processes of leukocytes meant to fight infections.

Basophilia is highly associated with CML ***

t(9;22)

Question 12

How to distinguish between Polycythemia Vera and reactive polycythemia?

Answer 12

Reactive polycythemia is a response to hypoxia or lung cancer.
So either there will be low SaO2 (saturation of oxygen) which results in increased EPO (erythropoiten).
Or there is ectopic EPO production of lung cancer with normal SaO2

In PV SaO2 is normal and EPO is decreased due to negative feedback

Question 13

Which myeloproliferative disorder does not have risk for hyperuricemia/gout and progression to acute leukemia?

Answer 13

Essential Thrombocytosis

Platelets are bits of megakaryocyte cytoplasm that break off. For example in PV, the nucelus of the RBC undergoes degredation into uric acid. No real nucelus waste in platelet production.

Question 14

PDGF (platelet derived growth factor) is what, what does it cause?

Answer 14

Proliferation of megakaryocytes, causing myelofibrosis

Question 15

Myelofibrosis place of production of RBCs

Answer 15

In the spleen (as opposed to marrow) resulting in splenomegaly

Question 16

myelofibrosis presents with leukoerythroblastic smear. What is it?

Answer 16

Bone marrow has Reticulin Gates, they prevent cells that are immature, and thus too big, from exiting into the blood. Since erythropoesis is in spleen, then you have white and red blasts in blood.

Question 17

Teardrop cells commonly seen in

Answer 17

Myelofibrosis

Some erythropoiesis still occurs in marrow, but cells get stretched when they squeeze out of fibrosed marrow.