hemolytic anemia
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Introduction
Defined as the premature destruction of erythrocytes with resultant anemia.
Etiology
- acquired
- hypersplenism
- immune-mediated hemolytic anemia
- IgG-mediated (extravascular hemolysis)
- IgM/complement-mediated (intravascular)
- drug-induced (may be IgG or IgM)
- alloimmune
- autoimmune
- paroxysmal nocturnal hemoglobinuria (intravascular)
- toxin & metabolic disorders
- arsenic-induced
- copper-induced
- thermal injury
- bacteria-induced
- protozoan infections:
- snake & spider bite - brown recluse
- hypophosphatemia
- spur cell hemolytic anemia (Zieve syndrome)
- parasitic infection (intravascular)
- erythrocyte trauma (extravascular, microangiopathic, schistocytes)
- hereditary hemolytic disorders
- membrane defects (extravascular)
- hereditary spherocytosis
- hereditary elliptocytosis (noted only with splenomegaly)
- enzyme defects
- thalassemia
- hemoglobinopathy - sickle cell disease & others
- idiopathic hereditary non-spherocytic hemolytic anemia
- overexpression of XPO7
- Wilson's disease
- membrane defects (extravascular)
Genetics
see hereditary hemolytic disorders above
History
- past medical history:
- family history:
Clinical manifestations
- symptoms
- symptoms common to all forms of anemia
- worsening of pre-existing angina
- heart failure - high output
- left upper quadrant pain secondary to splenomegaly
- right upper quadrant pain secondary to gallstones
- signs
- jaundice (intravascular)
- pallor
- cholelithiasis (intravascular)
- bilirubin gallstones especially common with chronic hemolysis
- splenomegaly (extravascular)
Laboratory
- complete blood count (CBC)
- anemia
- leukocyte & platelet count are sometimes elevated, but are usually normal
- MCV generally increased due to reticulocytosis[3]
- reticulocyte count is generally elevated
- peripheral smear - erythrocyte morphology
- direct antiglobulin test (Coomb's antibody)
- warm antibody autoimmune hemolytic anemia*
- IgG-positive direct antiglobulin (DAT) complement-C3 +/-[3]
- complement C3-positive, IgG negative DAT in cold agglutinin syndrome[3]
- complement positive Coomb's antibody in intravascular hemolysis
- IgG-positive direct antiglobulin (DAT) in extravascular hemolysis
- warm antibody autoimmune hemolytic anemia*
- serum lactate dehydrogenase
- increased in intravascular hemolysis
- normal in extravascular hemolysis
- serum bilirubin, unconjugated
- increased in intravascular hemolysis
- normal in extravascular hemolysis
- urinalysis: hemosiderinuria
- positive in intravascular hemolysis
- negative in extravascular hemolysis
- tests for prior or chronic hemolysis of several days
- serum haptoglobin
- decreased in intravascular hemolysis
- generally normal in extravascular hemolysis
- also an acute phase reactant; levels increased with inflammation
- elevated serum free hemoglobin in intravascular hemolysis
- hereditary spherocytosis
- osmotic fragility
- flow cytometry for eosin-5-maleimide binding to band-3 protein
- cold agglutinins - cold agglutinin syndrome
- hemoglobin electrophoresis
- serum glucose-6-phosphate dehydrogenase (serum G6PD)
- G6PD deficiency
- check 2-3 months after hemolytic episode[3]
- blood culture - if indicated
- paroxysmal nocturnal hemoglobinuria (PNH)
- see ARUP consult[5]
*schistocytes on peripheral blood smear sufficient to rule out warm autoimmune hemolytic anemia without direct antiglobulin testing (DAT)[1]
Diagnostic procedures
- echocardiography if hemolysis is suspected due to mechanical heart valve[3]
Complications
Differential diagnosis
- intravascular hemolysis
- extravascular hemolysis
- bleeding diathesis
- hypoplastic anemia
- primary marrow failure
- chronic renal insufficiency - decreased erythropoietin
Management
- supportive
- bedrest
- oxygen
- blood transfusion to treat symptoms, not Hgb/Hct
- aggressive treatment of cardiac/end organ hypoperfusion or ischemia
- treatment of underlying disorder (see underlying disorder)
- chronic hemolytic anemia (all patients)[3]
- chronic hemolytic anemia (strategies)[3]
More general terms
More specific terms
- autoimmune hemolytic anemia
- chronic nonspherocytic hemolytic anemia
- drug-induced hemolytic anemia
- Evan's syndrome
- Heinz body anemia
- hemoglobin C disease
- hemoglobin SC disease
- hemolytic anemia associated with ATPase deficiency
- hemolytic disease of the newborn
- hemolytic uremic syndrome (HUS)
- hereditary spherocytosis
- march hemoglobinuria; runner's anemia; exercise-induced hemolysis
- microangiopathic hemolytic anemia
- paroxysmal nocturnal hemoglobinuria (PNH, Marchiafava-Micheli syndrome)
- pyrimidine 5' nucleotidase deficiency hemolytic anemia; P5N deficiency
- regulator type Rh-null hemolytic anemia (Rh-deficiency syndrome)
- sickle cell (hemoglobin SS) disease
- thalassemia
Additional terms
- pharmaceutical agents associated with G6PD deficiency hemolytic anemia
- pharmaceutical agents associated with immune hemolytic anemia
References
- ↑ 1.0 1.1 Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 579-80
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2012, 2015, 2018.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ 4.0 4.1 Schiller G, in: UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- ↑ 5.0 5.1 ARUP Consult: ARUP Consult: Hemolytic Anemias The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/hemolytic-anemias
Hemolytic Anemias Testing Algorithm https://arupconsult.com/algorithm/hemolytic-anemias-testing-algorithm
Hereditary Hemolytic Anemia Panel, Sequencing https://arupconsult.com/ati/Hereditary-Hemolytic-Anemia-Panel - ↑ Shapira Y, Vaturi M, Sagie A. Hemolysis associated with prosthetic heart valves: a review. Cardiol Rev. 2009 May-Jun;17(3):121-4 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19384085
- ↑ Beris P, Picard V. Non-immune Hemolysis: Diagnostic Considerations. Semin Hematol. 2015 Oct;52(4):287-303. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26404441