Hematology + Oncology

Question 1

Give the associated pathology for the pathologic RBC: Ancanthocyte

Answer 1

Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)

Question 2

What is the “common name” for ancanthocytes?

Answer 2

Spur cells

Question 3

Give the associated pathology for the pathologic RBC: Basophilic stippling

Answer 3

Anemia of chronic disease, alcohol abuse, lead poisoning, thalassemias

Question 4

Give the associated pathology for the pathologic RBC: Bite cell

Answer 4

G6PD deficiency

Question 5

Give the associated pathology for the pathologic RBC: Elliptocyte

Answer 5

Hereditary elliptocytosis

Question 6

Give the associated pathology for the pathologic RBC: Macro-ovalocyte

Answer 6

Megaloblastic anemia (also hyper segmented PMNs), marrow failure

Question 7

Give the associated pathology for the pathologic RBC: Ringed sideroblast

Answer 7

Sideroblastic anemia. Excess iron in mitochondria = pathologic

Question 8

Give the associated pathology for the pathologic RBC: schistocyte, helmet cell

Answer 8

DIC, TTP/HUS, traumatic hemolysis (i.e mechanical heart valve prosthesis)

Question 9

Give the associated pathology for the pathologic RBC: sickle cell

Answer 9

Sickle cell anemia

Question 10

Give the associated pathology for the pathologic RBC: Spherocyte

Answer 10

Hereditary spherocytosis, autoimmune hemolysis

Question 11

Give the associated pathology for the pathologic RBC: teardrop cell

Answer 11

bone marrow infiltration (e.g., myelofibrosis)

Question 12

Give the associated pathology for the pathologic RBC: Target cell

Answer 12

HbC disease, Asplenia, Liver disease, Thalassemia

Question 13

Explain the pathological process associated with Heinz bodies

Answer 13

Oxidation of hemoglobin sulfhydryl groups –>denatured hemoglobin precipitation and phagocytic damage to RBC membrane –> bite cells.

Question 14

How can we visualize Heinz bodies?

Answer 14

crystal violet stain

Question 15

Explain the pathological process associated with Howell-Jolly bodies

Answer 15

Basophilic nuclear remnants found in the RBCs.

Howell-Jolly bodies are usually removed from RBCs by splenic macrophages

Question 16

List some pathologies associated with a finding of Howell-Jolly bodies

Answer 16

Functional hyposplenia

Asplenia

Question 17

List some pathologies associated with a finding of Heinz Bodies

Answer 17

See in G6PD deficiency

Heinz body like inclusions are seen in alpha-thalassemia

Question 18

What MCV value is consistent with microcytic anemias?

Answer 18

<80 fL

Question 19

What MCV value is consistent with normocytic anemia?

Answer 19

80-100 fL

Question 20

What MCV value is consistent with macrocytic anemia?

Answer 20

> 100 fL

Question 21

How are normocytic anemias further divided?

Answer 21

normocytic anemias are either non-hemolytic or hemolytic

Question 22

What is the reticulocyte count like in non-hemolytic normocytic anemias?

Answer 22

normal or decreased

Question 23

What is the reticulocyte count like in hemolytic anemias?

Answer 23

increased

Question 24

What pathologies lead to microcytic anemia? List 5

Answer 24

Iron Def (late)
ACD (anemia of chronic disease)
Thalassemias
Lead poisoning
Sideroblastic anemia

Question 25

What pathologies lead to non-hemolytic normocytic anemia? List 4

Answer 25

ACD (anemia of chronic disease) Aplastic anemia Chronic kidney disease Iron def (early)

Question 26

How are normocytic hemolytic anemias categorized?

Answer 26

Into intrinsic/extrinsic variations

Question 27

List 5 pathologies that end in intrinsic hemolytic anemia.

Answer 27

RBC membrane defect: hereditary spherocytosis RBC enzyme deficiency: G6PD, pyruvate kinase HbC defect Paroxysmal noctural hemoglobinuria Sickle cell anemia

Question 28

List 4 pathologies that end in extrinsic hemolytic anemia.

Answer 28

Autoimmune disease Microangiopathic Macroangiopathic Infections

Question 29

How are macrocytic anemias divided?

Answer 29

Into either magloblastic or non-megaloblastic

Question 30

List 3 pathologies that lead to megaloblastic macrocytic anemia.

Answer 30

Folate deficiency B12 def Orotic aciduria

Question 31

List 3 pathologies that lead to non-megaloblastic macrocytic anemia.

Answer 31

Liver disease Alcoholism Reticulocytosis

Question 32

What are the findings for microcytic, hypo chromic anemia secondary to iron deficiency?

Answer 32

``` Decreased Iron Increase total iron binding capacity Decreased ferritin fatigue conjunctival pallor May manifest as Plummer-Vinson syndrome ```

Question 33

Describe plummer-vinson syndrome

Answer 33

Triad of: Iron Def Esophageal webs Atrophic glossitis

Question 34

What is alpha-thalassemia?

Answer 34

A defect leading to alpha-globin gene deletions that result in decreased alpha-globin syn

Question 35

Which deletions do you see in alpha-thalassemia for the asian vs. african american population?

Answer 35

``` Cis = asian Trans = African ```

Question 36

Describe findings associated with a 3 allele deletion in alpha-thalassemia

Answer 36

HbH disease. Very little alpha-globulin Excess B-globin forms B4 (HbH)

Question 37

Describe the findings associated with a 1-2 allele deletion in alpha-thalassemia

Answer 37

no clinically significant anemia

Question 38

Describe the findings associated with a 4 allele deletion in alpha-thalassemia

Answer 38

No alpha-globulin Excess y-globulin forms Y4 (Hb Barts) Incompatible with life (causes hydrous fetalis)

Question 39

Describe microcytic, hypochromic anemia secondary to B-thalassemia

Answer 39

Point mutation in splice sites and promoter sequences --> decreased B-globin synthesis. (Prevalent in mediterranean populations)

Question 40

Describe the 3 findings associated with B-thalassemia minor (heterozygote)

Answer 40

- B chain is under produced - usually asymptomatic - diagnosis confirmed by increased HbA2 (>3.5%) on eletrophoresis

Question 41

Describe the 3 findings associated with B-thalassemia major (homozygous)

Answer 41

- B-chain is absent --> severe anemia requiring blood transfusion (secondary hemochomatosis) - marrow expansion --> skeletal deformaties. Chipmunk facies - Extramedullary hematopoiesis (leads to hepatosplenomegaly). Increased risk of parvovirus B19-induced aplastic crisis

Question 42

What significance does HbF play in infants?

Answer 42

HbF is protective in infants and disease doesn't become symptomatic until 6 mo.

Question 43

How does lead cause poisoning?

Answer 43

Lead inhibits ferrochelatase and ALA dehydratase --> decreased heme synthesis and increased RBC protoporphyrin. Also inhibits rRNA degredation, causing RBCs to retain aggregates of rRNA (basophilic stippling)

Question 44

List some signs of lead poisoning

Answer 44

- lead lines in the gingivae (burton lines) and on metaphases of long bones - encephalopathy and erythrocyte basophilic stippling - abdominal colic and sideroblastic anemia - drops: wrist and foot

Question 45

What is sideroblastic anemia?

Answer 45

defect in heme synthesis | hereditary: x-linked in gamma-ALA synthase gene

Question 46

What findings are associated with sideroblastic anemia?

Answer 46

ringed sideroblasts (Fe+ laden mitochondria) Increased Iron Normal total iron binding capacity increased ferritin

Question 47

List some causes of sideroblastic anemia.

Answer 47

Genetic Acquired (myelodysplastic syndromes) Reversible: alcohol, lead, vitamin B6 deficiency, copper deficiency, isoniazid

Question 48

restudy pages 384 onwards...

Answer 48

not making the rest of these freakin Q cards UGH!