Hemolytic Anemia with Extravascular Hemolysis Flashcards
Hemolytic Anemia with Extravascular Hemolysis
Hereditary includes?
Hemoglobinopathies (sickle cell anemia)
Enzymopathies (G6PD deficiency)
Membrane defects (hereditary spherocytosis)
Hemolytic Anemia with Extravascular Hemolysis
Acquired:
Immune Mediated?
Non-immune mediated
Autoimmune hemolytic anemia
non-immune mediated
Sickle Cell
Genitourinary complications
Hyposthenuria
Painless hematuria
Sickle Cell
Vaso-occlusive complications include?
Splenic infarction with hypoxia
Rhabdomyolysis or sudden death during extreme exertion
Sickle Cell Clinical Effects
Susceptible to infection
Functional Asplenia
Infarcted tissue
Numerous manipulations
Sickle Cell Clinical Effects
Chronic Hemolytic Anemia
Gallstones (calcium bilirubinate)
Risk of red cell aplasia (parvovirus)
Decreased vascular tone (low NO)
Sickle Cell Clinical Effects
Vaso-Occlusion
Microinfarction (vaso-occlusive crisis)
Acute Chest Syndrome
Priapism
Sickle Cell Precipitating Factors
Hypoxia
Acidosis
Fever
Infection
Dehydration
Exposure to cold
Sickle Cell: Acute Chest Syndrome Clinical Findings
Symptoms at diagnosis
Indistinguishable from PNA
Fever, Cough, Chest Pain, Tachypnea, SOB
Acute Chest Syndrome Lab Findings and Treatment
Lab diagnosis
worsening anemia
leukocytosis
decreased O2 sat
Acute Chest Syndrome Lab Findings and Treatment
CXR
Appears as PNA
Acute Chest Syndrome Lab Findings and Treatment
Treatment
Abx for CAP
Supplemental O2
RBC transfusions
Chronic Complications of Sickle Cell disease
Stroke
Avascular necrosis of hips and shoulders
CKD
Pulm HTN
Recommendations for Treatment in Sickle Cell Disease
Prevention includes what?
Disease-modifying therapy includes what?
Treatment of transfusion-related Iron overload?
Pneumococcus, meningococcus, H. Influenzae, and Hep B vaccines; PCN prophylaxis in infancy and childhood
RBC transfusion therapy; Hydroxyurea increases Hgb F
Chelation therapy for ferritin > 1000ng/ml
G6PD Deficiency
Clinical Lab findings include?
Chronic Hemolytic anemia
Bite Cells on peripheral smear
Coombs test negative
G6PD Deficiency
Treatment
Supportive Care
Stop Medication
Hereditary Spherocytosis
Clinical Lab findings
Splenomegaly
Chronic Hemolytic Anemia
Spherocytes on peripheral smear
Coombs test negative
Hereditary Spherocytosis
Treatment
Splenectomy
