Heme

Question 1

Causes of anemia in infants

Answer 1

Physiologic
Blood loss
Immune hemolytic disease
Congenital infection
Congenital hemolytic process

Question 2

Causes of anemia in kids

Answer 2

More likely acquired
Iron deficiency
Blood loss

Question 3

Causes/ labs for microcytic anemia

Answer 3

Iron deficiency or thalassemia
Low MCV count
Low RDW with iron deficiency

Question 4

Causes of normocytic anemia

Answer 4

Hemolytic anemia blood loss infection meds chronic disease

Question 5

Causes/labs in macrocyclic anemia

Answer 5

High MCV
Meds
B12 or folate deficiency
Hypothyroidism

Question 6

Aplastic anemia

Answer 6

Rare life threatening failure of hematopoesis leading to bone marrow aplasia and pancytopenia

Question 7

S/sx of pancytopenia

Answer 7

Bleeding parlor headache fatigue tachycardia petechia purpura echymosis jaundice and infection

Question 8

Lab findings of aplastic anemia

Answer 8

CBC shows pancytopenia
Low or no retic count
No evidence of malignancy

Question 9

Beta thalassemia

Answer 9

Chronic hemolytic anemia, iron overload and ineffective erythropoiesis

Question 10

Beta thalassemia presentation

Answer 10

Pallor irritability ftt diarrhea hepatosplenomegaly jaundice and bone deformities in the face
Severe microcytic hypochromic anemia with increased RBCs

Question 11

Tx of beta thalassemia

Answer 11

Blood transfusions very often

High risk of iron overload may need chelation

Question 12

G6PD deficiency triggers

Answer 12

Fava bean ingestion, infection, drug exposure

Question 13

G6PD presentation

Answer 13

Fever nausea abdominal pain diarrhea hemoglobinuria jaundice pallor tachycardia hepatosplenomegaly
Severe anemia increased wbc poikilocytes increased ret count

Question 14

Vado occlusive crisis s/sx

Answer 14

Tissue ischemia leads to severe pain and death of vascular beds
Dactylitis, chest head and back pain

Question 15

Management of vaso occlusive crisis

Answer 15

Adequate hydration, nsaids, opiods and encouraging adequate ventilation

Question 16

Anti microbial of choice in an acute infection with sickle cell

Answer 16

Penicillin, often already on prophylaxis

Question 17

Acute chest

Answer 17

Occurs in sickle cell disease

Ischemia/infarction of the lung, may develop pleural effusion

Question 18

Acute chest tx

Answer 18

Broad spectrum abx
Exchange or prbc infusion
Respiratory support

Question 19

When to screen for stroke in a sickle cell pt

Answer 19

Around age 2-16

*occurs in 7-10% of pts

Question 20

Splenic sequestration

Answer 20

Life threatening complication of sickle cell
Can lead to hypovolemic shock
Weakness pallor abdominal distension pain and splenimegaly

Question 21

Causes of DIC

Answer 21

Infection trauma ards ecmo and hematologist malignancy

Question 22

Lab findings in DIC

Answer 22

Thrombocytopenia prolonged Pt and ptt and prolonged d dimer*

Question 23

Hus triad

Answer 23

Thrombocytopenia hemolytic anemia and organ damage (often kidneys and brain)

Question 24

Most common infection in hus

Answer 24

Shiga toxin E. coli

Question 25

Hus symptoms

Answer 25

Abdominal pain watery/bloody diarrhea fever lethargy irritability and vomiting pallor petechiae hematites oliguria and hypertension

Question 26

ITP

Answer 26

Isolated thrombocytopenia with no other causes

Question 27

Henoch Schnlein Purpura presentation

Answer 27

Immune mediated small vessel vasculitis Rash, pain, swelling Hematuria proteinuria and hypertension diffuse abdominal pain Palpable purpura arthralgia acute arthritis

Question 28

Hemophilia

Answer 28

Hereditary x linked recessive chromosomal disease Factor 8 or 9 deficiency Clotting disorder

Question 29

Hemophilia tx/prevention

Answer 29

Replace missing clotting factor Prbc ffp and cryo transfusions Ddavp for moderate bleeding Education on prevention

Question 30

Von willebrand

Answer 30

Deficiency in vonwillebrand protein | Often diagnosed later in life

Question 31

Most sensitive study for embolic disorders

Answer 31

CT agnio

Question 32

Why to give ffp

Answer 32

Replaces coagulation factors | Dose 10-15 ml/kg

Question 33

Why to give cryo

Answer 33

Contains fibrinogen factor 8, 13 vonwillebrand factor and fibronectin Given when fibrinogen is < 100 Give 1 unit per 5-10 kg of body weight

Question 34

Diagnostic for sickle cell anemia

Answer 34

Hemoglobin electropheresis

Question 35

Acute hemolytic reaction

Answer 35

Transfusion reaction related to hemolysis secondary to ABO incompatibility Fever chills lumbar pain shock dyspnea hemoglobinuria chest pain DIC

Question 36

TACO

Answer 36

Circulatory overload post transfusion | Resp distress, hypoxia hypertension

Question 37

TRALI

Answer 37

Immune response post transfusion that leads to alveolar injury 6 hours post