Exam 2 - Anemia

Question 1

Phagocyte Function

Answer 1

1. Rolling/Adherence
2. Chemotaxis
3. Ingestion
4. Degranulation/Killing
   CD11b/CD18 are important receptors for several steps

Question 2

Leukocyte Adhesion Deficiency I
Clinical Presentation
Functional Deficit
Molecular Deficit

Answer 2

CP: recurrent soft tissues infections (mouth), poor wound healing
FD: Neutrophilia, decreased adherence to endothelial surfaces
MD: CD18 deficient, can’t express CD11b/CD18
Autosomal recessive

Question 3

Chediak-Higashi Syndrome
Clinical Presentation
Functional Deficit
Molecular Deficit

Answer 3

CP: albinism, recurrent infection, fever, hepatosplenomegaly, neurodegeneration
FD: Neutropenia, giant granules in leukocytes, abnormal/decreased degranulation
MD: Giant, leaky granules, altered MT assembly, alterations in membrane fusion
Autosomal recessive

Question 4

Myeloper-oxidase Deficiency
Clinical Presentation
Functional Deficit
Molecular Deficit

Answer 4

CP: Generally healthy, increased fungal infections w/ diabetes
FD: myeloperoxidase deficiency, mild killing bacteria, can’t kill candida
MD: Post-translation defect in processing protein
Autosomal recessive

Question 5

Chronic Granulomatous Disease
Clinical Presentation
Functional Deficit
Molecular Deficit

Answer 5

CP: recurrent purulent infections w/ catalase positive, deep infections of lung, liver, spleen, LN, and bone
FD: Neutrophilia. Normal activation, defect in oxidase - no toxic metabolites
MD: Defect in:
cyto b558/gp91phox - X linked recessive
p22phox or p47phox or p67phox - autosomal recessive
mild G6PD deficency - X linked variant

Question 6

Complement Factors and Deficiencies:
C1q, C2, C4
C3
C5-C9

Answer 6

1,2,4: SLE, autoimmunity, inflammatory vascular dz
3: recurrently bacterial infections
5-9: Neisseria infections

Question 7

Management of Innate Immune Disorders

Answer 7

1. Anticipation of infections
2. Surgical procedures (dx and tx)
3. Prompt broad spectrum abx, followed with specific
4. Severe: G-CSF to achieve adequate ANC
5. Prophylactic abx or cytokines (INF-gamma)

Question 8

Storage pool vs peripheral blood pool

Answer 8

Storage pool: blood conc. x 20

Peripheral blood pool: blood conc. x 1

Question 9

Neutropenia

Answer 9

Decrease in neutrophils (incl bands and segs)

Question 10

Risk of infection

assume blood levels correspond to marrow pool

Answer 10

ANC 1000-1500 (none)
ANC 500-1000/nml (mild)
ANC

Question 11

Neutropenic Disorders (Primary or Secondary)

Answer 11

Kostmann’s
Cyclic Neutropenia
Shwachman-Diamond
Idiopathic Neutropenia

Question 12

Neutropenic Disorders (Normal reserve, increased turnover)

Answer 12

Infection
Drug
Antibody associated
Cancer/chemo (other cell lines down)
Hypersplenism (other cell lines down)
Aplastic anemia (other cell lines down)
B12/Folate deficiency (other cell lines down)

Question 13

Microcytic Anemia

Answer 13

Fe Deficiency
Anemia of chronic dz
Pb intoxication
Hereditary spherocytosis (hemolytic)
Thalassemia (hemolytic)

Question 14

Macrocytic Anemia

Answer 14

B12 Deficiency

Folate Deficiency

Question 15

Hemolytic Anemia

Answer 15

G6PD Deficiency
Autoimmune hemolytic anemia
PK Deficiency

Question 16

Anemia of chronic dz (inflammation)

Answer 16

Low TIBC

Normal ferritin

Question 17

Response to Epo injections

Answer 17

Increased RC
Fall in MCV if iron deficiency is unmasked
Polycythemia (Hct >52%) if hypoxia is present

Question 18

Normocytic Anemia

Answer 18

Sickle cell
Renal failure
Autoimmune hemolytic (variable)
Sideroblastic

Question 19

High retic count seen in:

Answer 19

Sickle cell anemia
Recovery from acute, severe bleeding
Hemolysis (HS, or AIHA)

Question 20

Warm antibody AIHA

Answer 20

IgG
High affinity to RBC membrane
Little complement
Extravascular hemolysis

Question 21

Cold antibody AIHA

Answer 21

IgM
Low affinity to RBC membrane
Activates complement
Intravascular hemolysis