Disorders Of WBC and RBC

Question 1

The primary chemotherapeutic drug for ALL? Side effect?

Answer 1

Asparginase (Pancreatitis)

Question 2

Used as delivery vehicle to sanctuary sites of ALL?

Answer 2

Ommaya Reservoir

Question 3

Most common cancer in children?

Answer 3

Acute lymphoblastic Leukemia

Question 4

Leukemia in adults 15-39 y.o?

Answer 4

AML

Question 5

Distinctive peroxidase positive, needle like azurophilic granules present in AML?

Answer 5

Auer rods

Question 6

Caused by translocation t15:17?

Answer 6

Acute promyelocytic leukemia

Question 7

Acute promyelocytic leukemia is highly responsive to what vitamin?

Answer 7

All-trans retinoic acid

Question 8

Associated with t9:22; Philadelphia chromosome

Answer 8

Chronic Myelogenous leukemia (BCR-ABL fusion gene)

Question 9

Scattered macrophages with abundant wrinkled, green blue cytoplasm? Seen in what condition?

Answer 9

Sea-blue histiocytes. CML

Question 10

WHO criteria for Blast crisis?

Answer 10

1. > 20% myeloblast or lymphoblast in blood or BM
2. Large cluster of blasts in BM biopsy
3. Development of Chloroma

Question 11

What is the chemotherapeutic drug of choice for CML? Side effects?

Answer 11

Imatinib

Fluid retention, CHF

Question 12

Most common leukemia of adults and elderly?

Answer 12

Chronic Lymphocytic Leukemia

Question 13

Differentiate CLL from SLL.

Answer 13

CLL if absolute lymphocyte count > 4000

SLL if absolute lymphocyte count is

Question 14

Small lymphocytes disrupted in the process of making smears? Characteristic of what condition?

Answer 14

Smudge cells. CLL

Question 15

Transformation of CML into diffuse large B cell lymphoma

Answer 15

Ritcher syndrome

Question 16

The only NHL associated with EBV infection?

Answer 16

Burkitt’s Lymphoma

Question 17

These are large cells with multiple or single nuclei with multiple nuclear lobes and each half a mirror image of the other? What condition?

Answer 17

Reed-Sternberg Cells in Hodgkin’s lymphoma

Question 18

The type of Hodgkin’s Lymphoma not associated with EBV?

Answer 18

Nodular Sclerosis

Lymphocytic Predominant type

Question 19

Type of Hodgkin’s lymphoma with a poor prognosis?

Answer 19

Lymphocyte depleted type

Question 20

Type of Hodgkin’s Lymphoma characterized with nodular infiltrate of small lymphocytes admixed with macrophages? Characteristic cells?

Answer 20

Lymphocyte- Predominant

Popcorn cells

Question 21

Condition associated with t14;18 involving the BCL2?

Answer 21

Follicular Lymphoma

Question 22

The most common form of NHL?

Answer 22

Diffuse Large B-cell Lymphoma

Question 23

Associated with t8;14?

Answer 23

Burkitt’s Lymphoma

Question 24

Characteristic morphologic pattern seen in Burkitt’s lymphoma?

Answer 24

Starry sky pattern

Question 25

Associated with t11;14?

Answer 25

Mantle cell Lymphoma

Question 26

Neoplasm of CD4+ T cells caused by HTLV-1? Characteristic cell?

Answer 26

Adult T-cell Lymphoma | Cloverleaf or Flower cells

Question 27

Diffuse erythema and scaling of the entire body associated with Mycosis Fungiodes?

Answer 27

Sezary Syndrome

Question 28

Excess light or heavy chains with complete Igs synthesized by neoplastic plasma cells that are excreted in the urine?

Answer 28

Bence- Jones Proteins

Question 29

Mutiple Myeloma Morphology

Answer 29

``` Plasmacytoma (punched out lesions) Flame cells Mott cells Russell bodies (pink) Dutcher bodies (blue) Rouleaux conformation (stack of coins) ```

Question 30

Hallmark of Primary Myelofibrosis.

Answer 30

Development of obliterative marrow fibrosis

Question 31

Pentalaminar tubules with dilated terminal ends (tennis-racket) containing langerin protein? What condition?

Answer 31

Birbeck granules in Langerhans Cell Histiocytosis

Question 32

Type of hemolysis wherein the defects are inherent to the RBCs.

Answer 32

Intrinsic Hemolysis

Question 33

Differentiate intravascular from extravascular hemolysis.

Answer 33

Extravascular has Splenomegaly

Question 34

Small hyperchromic RBC laking central pallor.

Answer 34

Spherocytes

Question 35

Caused by mutations of ankyrin, band 3, spectrin and band 4.2 which renders the RBCs less deformable?

Answer 35

Hereditary Spherocytosis

Question 36

Presents with prominent reticulocytosis in the peripheral blood.

Answer 36

Hemolytic anemia

Question 37

Small dark nuclear remnants present in RBCs of asplenic patients. What condition?

Answer 37

Howell-Jolly Bodies | Hereditary spherocytosis

Question 38

This is a condition wherein there is reduced ability of RBCs to protect themselves from oxidative injuries leading to hemolysis.

Answer 38

G6PD deficiency

Question 39

G6PD is the rate limiting step of which biochemical pathway? Function of enzyme?

Answer 39

Pentose Phosphate pathway | Reduces NAD to NADPH

Question 40

1. Membrane-bound precipitates on denatured globin chains. 2. RBC with damaged membranes due to removal of no 1 by splenic macrophages What condition?

Answer 40

1. Heinz bodies 2. Bite cells G6PD deficiency

Question 41

Pathogenesis of sickle cell anemia.

Answer 41

Point mutation at 6th codon of B-globin ( replacement of Glutamate with Valine)

Question 42

These are dehydrated RBC cells with bull's eye apperance. What condition?

Answer 42

Target cells | Sickle cell anemia; Thalassemia

Question 43

A condition characterized by chipmunk facies and crewcut skull apperance.

Answer 43

Sickle cell anemia | Thalassemia

Question 44

What is the initial diagnostic text for Sickle cell anemia? Gold standard test?

Answer 44

Peripheral Blood smear | Hemoglobin electrophoresis

Question 45

What is the drug of choice for sickle cell anemia?

Answer 45

Hydroxyurea (increases HbF)

Question 46

Type of Alpha Thalassemia characterized by deletion of 3 alpha-globin genes.

Answer 46

Hemoglobin H disease

Question 47

The most sever form of Alpha thalassemia?

Answer 47

Hydrops fetalis (HbBarts)

Question 48

A disease of RBCs wherein it completely lacks HbA leading to transfusion dependence.

Answer 48

Beta-Thalassemia Major (Cooley's anemia)

Question 49

Morphology of anemia present in Thalassemia.

Answer 49

Microcytic, hypochromic anemia

Question 50

Treatment strategy for patient unresponsive to primary treatment of Hemochromatosis.

Answer 50

Chelation with deferoxamine

Question 51

A condition characterized with abnormally large erythroid precursors and red cells. This is due to what vitamin deficiency?

Answer 51

Megaloblastic Anemia | Vitamin B12/ Folate deficiency

Question 52

Which parasitic infection leads to megaloblastic anemia? What is the drug of choice?

Answer 52

Diphyllobothrium Latum infection | Praziquantel

Question 53

Test to diagnose pernicious anemia.

Answer 53

Schilling test

Question 54

What is the most common nutritional disorder in the world?

Answer 54

Iron deficiency anemia

Question 55

Which parasitic infection leads to microcytic, hypochromic anemia? What is the drug of choice?

Answer 55

Hookworms | Albendazole

Question 56

Pathophysiology in anemia of chronic disease.

Answer 56

IL-6 stimulates increase in HEPCIDIN which reduces iron transfer from storage pool to BM

Question 57

Condition characterized morphologically as hypocellular BM with fat cells.

Answer 57

Aplastic Anemia

Question 58

An X-linked defect in D-Ala synthase gene causing defect in heme synthesis.

Answer 58

Sideroblastic anemia

Question 59

What is the most feared complication of ITP?

Answer 59

Intracranial Bleeding

Question 60

A condition characterized by decreased gp Ib leading to defective platelet adhesion and decreased platelet count.

Answer 60

Bernard-Soulier Syndrome

Question 61

What happens in Glanzmann's Thrombasthenia?

Answer 61

Decrease in gp IIb-IIIa leadig to defective platelet aggregation with normal platelet count.

Question 62

Primary treatment for von Willebrand Disease?

Answer 62

Desmopressin

Question 63

Hemophilia A is due to what factor deficiency?

Answer 63

Factor VIII

Question 64

A bleeding disorder due to deficient factor IX?

Answer 64

Hemophilia B/ Christmas Disease

Question 65

What is the main protective mechanism against mercury poisoning?

Answer 65

Intracellular Glutathione