Heme Flashcards
Causes of anemia in infants
Physiologic Blood loss Immune hemolytic disease Congenital infection Congenital hemolytic process
Causes of anemia in kids
More likely acquired
Iron deficiency
Blood loss
Causes/ labs for microcytic anemia
Iron deficiency or thalassemia
Low MCV count
Low RDW with iron deficiency
Causes of normocytic anemia
Hemolytic anemia blood loss infection meds chronic disease
Causes/labs in macrocyclic anemia
High MCV
Meds
B12 or folate deficiency
Hypothyroidism
Aplastic anemia
Rare life threatening failure of hematopoesis leading to bone marrow aplasia and pancytopenia
S/sx of pancytopenia
Bleeding parlor headache fatigue tachycardia petechia purpura echymosis jaundice and infection
Lab findings of aplastic anemia
CBC shows pancytopenia
Low or no retic count
No evidence of malignancy
Beta thalassemia
Chronic hemolytic anemia, iron overload and ineffective erythropoiesis
Beta thalassemia presentation
Pallor irritability ftt diarrhea hepatosplenomegaly jaundice and bone deformities in the face
Severe microcytic hypochromic anemia with increased RBCs
Tx of beta thalassemia
Blood transfusions very often
High risk of iron overload may need chelation
G6PD deficiency triggers
Fava bean ingestion, infection, drug exposure
G6PD presentation
Fever nausea abdominal pain diarrhea hemoglobinuria jaundice pallor tachycardia hepatosplenomegaly
Severe anemia increased wbc poikilocytes increased ret count
Vado occlusive crisis s/sx
Tissue ischemia leads to severe pain and death of vascular beds
Dactylitis, chest head and back pain
Management of vaso occlusive crisis
Adequate hydration, nsaids, opiods and encouraging adequate ventilation
Anti microbial of choice in an acute infection with sickle cell
Penicillin, often already on prophylaxis
Acute chest
Occurs in sickle cell disease
Ischemia/infarction of the lung, may develop pleural effusion
Acute chest tx
Broad spectrum abx
Exchange or prbc infusion
Respiratory support
When to screen for stroke in a sickle cell pt
Around age 2-16
*occurs in 7-10% of pts
Splenic sequestration
Life threatening complication of sickle cell
Can lead to hypovolemic shock
Weakness pallor abdominal distension pain and splenimegaly
Causes of DIC
Infection trauma ards ecmo and hematologist malignancy
Lab findings in DIC
Thrombocytopenia prolonged Pt and ptt and prolonged d dimer*
Hus triad
Thrombocytopenia hemolytic anemia and organ damage (often kidneys and brain)
Most common infection in hus
Shiga toxin E. coli
Hus symptoms
Abdominal pain watery/bloody diarrhea fever lethargy irritability and vomiting pallor petechiae hematites oliguria and hypertension
ITP
Isolated thrombocytopenia with no other causes
Henoch Schnlein Purpura presentation
Immune mediated small vessel vasculitis
Rash, pain, swelling
Hematuria proteinuria and hypertension diffuse abdominal pain
Palpable purpura arthralgia acute arthritis
Hemophilia
Hereditary x linked recessive chromosomal disease
Factor 8 or 9 deficiency
Clotting disorder
Hemophilia tx/prevention
Replace missing clotting factor
Prbc ffp and cryo transfusions
Ddavp for moderate bleeding
Education on prevention
Von willebrand
Deficiency in vonwillebrand protein
Often diagnosed later in life
Most sensitive study for embolic disorders
CT agnio
Why to give ffp
Replaces coagulation factors
Dose 10-15 ml/kg
Why to give cryo
Contains fibrinogen factor 8, 13 vonwillebrand factor and fibronectin
Given when fibrinogen is < 100
Give 1 unit per 5-10 kg of body weight
Diagnostic for sickle cell anemia
Hemoglobin electropheresis
Acute hemolytic reaction
Transfusion reaction related to hemolysis secondary to ABO incompatibility
Fever chills lumbar pain shock dyspnea hemoglobinuria chest pain DIC
TACO
Circulatory overload post transfusion
Resp distress, hypoxia hypertension
TRALI
Immune response post transfusion that leads to alveolar injury
6 hours post
