Hematology

Question 1

List the differential for splenomegaly

Answer 1

Portal Hypertension:
- liver failure
- congestive heart failure
Hematologic:
- acute or chronic hemolytic anemia
- sickle cell
Infection
- viral (hepatitis, infectious mononucleosis, cytomegalovirus)
- bacterial
- parasitic
Inflammatory:
- SLE
Infiltrative
- glycogen deposition disease
- leukemia
- lymphoma

Question 2

Discuss the differential for polycythemia

Answer 2

Relative:
- decreased plasma volume (low hematocrit, dehydration)
Primary
- acquired: polycythemia Vera
- hereditary
Secondary
- acquired: hypoxemia (pulmonary disease, right to left shunt, high altitude), carboxyhemoglobinemia (smoking, CO poisioning), endogenous erythropoietin (tumours, polycystic kidney)
- hereditary: high affinity hemoglobinopathies, congenital methhemoglobinemia

Question 3

Discuss the diagnostic approach to polycythemia Vera

Answer 3

Low serum EPO suggest primary polycythemia
- JAK2 mutation and bone marrow study to rule out polycythemia Vera
High Serum EPO suggest secondary cause
- look at if hypoxemic or have chronic exposures
- very high suggest tumour
- family history

Question 4

List the differential for normocytic (80-100) anemia

Answer 4

Low reticulocyte count:
- anemia of chronic disease
- bone marrow failure: myelofibrosis, aplastic anemia
High reticulocyte count:
- hemolytic anemia:
- inherited: hemoglobinopathies (sickle cell, thalassemia) membrane (elliptocytosis, spherocytosis)
- acquired: immune (Coombs positive, drug related), malaria, DIC, TTP
- acute hemorrhage
- chronic renal failure
- hypothyroidism

Question 5

List the differential diagnosis for anemia by age

Answer 5

Neonatal:
- hemorrhage
- isoimmunization (ABO, Rh)
- congenital hemolytic anemia (spherocytosis, G6PD)
- TORCH
Infant:
- iron deficiency
- genetic hemolytic anemia 
- GI bleed
- leukemia 
Child/Adolescence:
- iron deficiency
- blood loss
- hemoglobinopathies
- leukemia

Question 6

Discuss the presentation of anemia

Answer 6

• Asymptomatic
• Behavioural: pica, irritability, lethargy
• Jaundice
• Syncope, dyspnea
• Pallor
• Palpitations
• Failure to thrive

Question 7

When would a bone marrow biopsy be performed

Answer 7

When child was >=2 of:

• anemia
• leukopenia
• thrombocytopenia

Question 8

Discuss the transfusion guidelines for anemia

Answer 8

<4 Months:
- HCT >45% on ECMO or CHD
- HCT <30% with unstable vitals or require oxygenation
- HCT <20% with symptoms and low reticulocyte count]
>4 Months:
- Hbg <70

Question 9

Discuss the difference between platelet disordered bleeding and coagulopathies

Answer 9

Platelets:

• skin petechiae
• small, scattered ecchymoses
• bleed from superficial skin abrasions
• predominantly in females

Coagulopathies

• larger ecchymoses
• soft tissue hematoma
• hemarthrosis
• delayed bleeding
• family history of bleeding
• predominantly male

Question 10

Discuss the laboratory findings of alpha and beta thalasemia minor

Answer 10

Alpha:
- low MCV
- target cells
- normal or increased iron and ferritin
- normal electrophoresis
beta
- low MCV
- target cells
- normal or increased iron and ferritin
- increased hemoglobin A2
- major and intermedia require blood transfusions

Question 11

What are the dysmorphic features found in thalassemia major

Answer 11

• frontal and cheek bossing
• saddle nose
• maxillary protrusion (chipmunk face)