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Anemia (222)
Eric Quimbo, DO
June 8, 2006
Introduction
Destruction of red blood cells
(RBCs) not due to genetic or
congential disorder
Varying disease severity
Asymptomatic to fatal hemolysis
Presentation
Weakness
Dizziness
DOE
Tachycardia
Murmur
Abdominal pain
Pallor
Jaundice
Fever,
lymphadenopathy
systemic disease
Splenomegaly
Chronic
Hepatomegaly
Darkened urine
Intravascular
hemolysis,
hemoglobinuria
Diagnosis
Spherocytes/schistocytes on
peripheral smear
Hct
Retic count
indirect bilirubin ( Hgb breakdown)
LDH
http://www.wadsworth.org/chemheme/heme/glass/cytopix/slide014schisto2.jpg
Overview
Immune-mediated
Autoimmune
Alloimmune
Hemolytic disease of the newborn
Drug-related
Microangiopathic
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic-uremic syndrome (HUS)
Pregnancy-related (HELLP)
Macrovascular
Prosthetic heart valves
Autoimmune Hemolytic
Anemia (AIHA)
Result of generalized immune
system dysfunction
1-3 cases/100,000 yearly
Diagnosis
Positive direct antigen test (DAT) and
antibody identification
Types
Warm Antibody
Cold Antibody
Autoimmune Hemolytic
Anemia (AIHA)
Warm Antibody (near 37 C)
70% AIHA cases
Chronic lymphocytic leukemia
Treatment
First line
High-dose corticosteroids prednisone PO 1 1.5 mg/kg QD for 13 weeks
Splenectomy
Removes site of hemolysis and of antibody production
65 70% response rate
Should receive pneumococcal and meningiococcal vaccinations b/c
of risk of infection of encapsulated bacteria
Cytotoxic Drugs
40 60% response rate
Failed steroid and splenectomy treatment
More AIHA
Cold Antibody
Most hemolytic at 0 4 C, leading to
clumping of RBCs on the smears
Cold Agglutinin Syndrome (CAS)
Typically mild symptoms
Hgb 9 12
Tx avoid cold weather, symptomatic tx
Microangiopathic
Syndromes
Involve platelet aggregation in the
microvascular circulation via mediation of
von Willebrand factor (vWF) leading to
thrombocytopenia
Microangiopathic anemia (MAHA)
develops as fragmented RBCs travel
through occluded capillaries
Types
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic Uremic Syndrome
www.academic.marist.edu/.../description31.html
Clinical Findings
TTP
Adults
Neuro effects
HUS
Children
Renal system
impairment
Thrombotic
Thrombocytopenic Purpura
(TTP)
Classic Pentad
Thrombocytopenia
MAHA
Fever
Renal impairment
Neurologic impairment
Microthrombi created from the large vWF multimers occur throughout the
systemic circulation
Triggers pregnancy, infection, autoimmune disorders
Diagnosis PLT < 20K, schistocytes on smear, elevated retic count
Treatment
Plasma exchange
Corticosteroids
Splenectomy
**Platelet transfusions should be avoided unless life-threatening bleeding or
ICH
http://dermatlas.med.jhmi.edu/derm/display.cfm?ImageID=1223608648
Hemolytic Uremic
Syndrome
ARF
MAHA
http://www.dental.mu.edu/oralpath/lesions/petechia/petechia2.jpeg
Fever
Thrombocytopenia
Clinical - restless, irritable, petechiae, purpura and oozing
may occur
HELLP Syndrome
Complication of preeclampsia
Hemolysis, elevated liver enzymes, low platelets
1/1000 pregnancies
Microvascular platelet-fibrin thrombi which
initially predominate in the liver and may spread
Decreased serum haptoglobin is most sensitive
marker
Treatment delivery of baby
Supportive measures if unable to deliver immedately
THE
END!
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