Hematology

Question 1

What pathology is associated with this pathologic RBC disease: Lead poisoning

Answer 1

Basophilic Stippling

Question 2

What pathology is associated with this pathologic RBC disease: G6PD Deficiency

Answer 2

Degmacyte (“bite cell”)

Question 3

What pathology is associated with this pathologic RBC disease: Hereditary elliptocytosis

Answer 3

Elliptocyte

Question 4

What pathology is associated with this pathologic RBC disease: Megaloblastic anemia (hypersegmented PMNs), marrow failure.

Answer 4

Macro-ovalocyte

Question 5

What pathology is associated with this pathologic RBC disease: Sideroblastic anemia. Excess iron in mitochondria=pathologic

Answer 5

Ringed sideroblast

Question 6

What cell type/shape is associated with this pathologic RBC disease: Microangiopathic hemolytic anemias, DIC, TTP/HUS, HELLP syndrome, mechanical hemolysis (e.g. heart valve prosthesis). —> fragmented RBCs

Answer 6

Schistocyte (“helmut cell”)

Question 7

What pathology is associated with this pathologic RBC disease: Sickling of cells can occur during times of dehydration, deoxygenation, and at high altitude.

Answer 7

Sickle cell

Question 8

What cell type and pathology is associated with this pathologic RBC disease: small spherical cells without central pallor, drug-and infection-induced hemolytic anemia.

Answer 8

Spherocytes + Hereditary Spherocytosis

Question 9

What pathology is associated with this pathologic RBC disease: Bone marrow infiltration (e.g., myelofibrosis). RBC “sheds a tear” because it’s mechanically squeezed out of its home in the bone marrow.

Answer 9

Dacrocyte (“teardrop cell”)

Question 10

What pathology is associated with this pathologic RBC disease: HbC disease, Asplenia, Liver disease, Thalassemia.

Answer 10

Target cell (RBC looks like Target sign).

Question 11

What cell type is associated with this pathologic RBC disease: Seen in G6PD deficiency; ______ inclusions seen in alpha-thalassemia. –> Oxidation of Hb-SH groups to S-S- –> Hb Precipitation, with subsequent phagocytic damage to RBC membrane –> ____ cells caused by spleen.

Answer 11

Heinz Bodies and

Bite cells

Question 12

What pathology is associated with this pathologic RBC disease: Basophilic nuclear remnants found in RBCs. Seen in Px with functional hyposplenia or Asplenia.

Answer 12

Howell-Jolly bodies - They are normally removed from RBCs by splenic macrophages.

Question 13

Coagulation Disorders: What is the function of a PT test? Which coagulation factors does it involve?

Answer 13

Involves factors I, II, V, VII, and X.

–> A defect would increase PT (because clotting cannot occur).

Question 14

Coagulation disorders: PTT is a function to test what? What clotting factors does it involve?

Answer 14

PTT tests function of common and intrinsic pathway (all factors except VII and XIII). A defect would lead to increased PTT (blood is unable to clot).

Question 15

Hereditary syndromes leading to hypercoagulability: Inherited deficiency of antithrombin: has o direct effect on the PT, PTT, or thrombin time but diminishes the increase in PTT following heparin administration. Can also be acquired: renal failure/nephrotic syndrome –> antithrombin loss in urine –> decrease inhibition of factors IIa and Xa.

Answer 15

Antithrombin deficiency

Question 16

These cells are distinctive tumor giant cells seen in Hodgkin disease; binucleate or bilobed with the 2 halves as mirror images (owl eyes). RS cells are CD15+ and CD30+ B-cell origin. Lymphocyte-rich form has best prognosis. Lymphocyte mixed or depleted forms have worse prognosis.

Answer 16

Reed-Sternberg cell

Question 17

What pathology is associated with this pathologic RBC disease: Liver disease, abetalipoproteinemia (states of cholesterol dysregulation).

Answer 17

Acanthocyte (“spur cell”)

Question 18

Associated Translocations: 8;14

Answer 18

Burkitt Lymphoma

Question 19

Associated Translocations: t(15;17)

Answer 19

M3 type of AML

Treatment is with all Trans-Retinoic Acid

Question 20

Associated Translocations: t(8;21)

Answer 20

AML

Question 21

What condition is associated with a defect in the following enzyme: Uroporphyrinogen decatboxylase

Answer 21

Porphyria cutanea tarda–> looks like homeless man!

Blisters with photosensitivity, etc.

Question 22

What condition is associated with a defect in the following enzyme: porphobilinogen deaminase aka: uroporphyrinogen-1-synthase

Answer 22

Acute intermittent porphyria

5Ps: painful abdomen
Port-wine colored urine
Polyneuropathy 
Psychological disturbances 
Precipitated by drugs: cytochrome P-450 inducers, alcohol, rifampin, metoclopramide, and starvation.

Question 23

What is the most common type of Non-Hodgkin Lymphoma in adults and the US? What is the associated translocation?

Answer 23

Diffuse Large B Cell Lymphoma

t(14;18)

Question 24

What is the associated translocation in Mantle Cell Lymphoma? What portion of the population is it more associated with?

Answer 24

T(11;14) - older males CD5+

Question 25

What type of neoplasm is associated with long term celiac disease?

Answer 25

Adult T-Cell Lymphoma caused by HTLV (IV drug abuse) --> lyric bone lesions, hypercalcemia

Question 26

What disease is at hand: adenosine deaminase deficiency?

Answer 26

SCID - severe combined immunodeficiency disorder

Question 27

What disease is at hand: Neutrophils fail to respond to chemotactic stimuli?

Answer 27

Could be either: Job Syndrome (hyper IgE syndrome) OR Leukocyte adhesion deficiency syndrome

Question 28

What is a Kupffer cell?

Answer 28

It is a Macrophage that is found in the liver.

Question 29

What is a histiocyte?

Answer 29

It is a Macrophage that is found in Connective Tissue.

Question 30

What is a Langerhans cell?

Answer 30

It is a Macrophage that is found in the Skin!

Question 31

What is a microglial cell?

Answer 31

It is a Macrophage that is found in the Brain

Question 32

What is an osteoclast and what does it do?

Answer 32

Basically a macrophage that is found in bone! It also absorbs bone tissue during growth and healing.

Question 33

What are some causes of Eosinophilia (PACCMAN)?

Answer 33

``` Parasites Asthma Churg-Strauss Syndrome Chronic adrenal insufficiency Myeloproliferative disorders Allergic Process Neoplasia (eg Hodgkin Lymphoma) ```

Question 34

What is the MOA of Warfarin?

Answer 34

Inhibits epoxide reductase —> responsible for recycling Vitamin K Vit K is needed for creating Vit k dependent clotting factors.

Question 35

What drug is used to reverse the effects of Heparin?

Answer 35

Protamine sulfate

Question 36

What is the rescue drug for Warfarin overdose?

Answer 36

Vitamin K or Fresh frozen plasma

Question 37

What are the three most common Thrombolytic drugs? What is the MOA of these drugs?

Answer 37

Streptokinase Urokinase tPA (alteplase) RESCUE: Aminocaproic Acid They activate plasmin —> plasmin degrades fibrin (clot) C/I in Px with active bleeding, Hx of intracranial bleeding, severe hypertension, recent surgery, or an unknown bleeding disorder.

Question 38

What are the 3 main functions of Bradykinin?

Answer 38

- Vasodilator - Increases vascular permeability - Mediator of pain

Question 39

What kind of RBCs are seen in asplenic patients??

Answer 39

- Howell-Jolly bodies | - Target Cells

Question 40

What is the rate limiting enzyme for heme synthesis?

Answer 40

Aminolevulinic acid synthase Made from Glycine and succinyl-CoA —> and make aminolevulinic acid with the help of Vit. B6

Question 41

48 y/o male presents with blistering of the skin from photosensitivity, tea colored urine, hypertrichosis, facial pigmentation, has HEP C, elevated LFTs, and is an alcoholic. What is the most likely diagnosis?

Answer 41

Porphyria cutánea tarda

Question 42

What is the pathology at hand: Dysplasia of the hematopoietic cells in the myeloid tissue and disordered hematopoiesis. On peripheral blood smear, neutrophils have nuclei with 2 loves that are connected by a thin strand (like poncinot glasses worn by Rosevelt). What is this finding called as well.

Answer 42

Myelodysplastic Syndrome | (Pre-leukemia) and the blood smear shows “Pelger-Huet Anomaly”