Acquired Hemolytic

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

Can be isolated or part of a large

clinical syndrome

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

RBC Transfusion (use the least incompatible)

Life-threatening anemia
High-risk cardiac or cerebrovascular

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

Paroxysmal Cold Hemoglobinuria (PCH)

Hemolysis at cold & normal temps
High fever, chills, abdominal pain with cold weather
Tx keep warm, consider steroids
http://www.meddean.luc.edu/lumen/MedEd/medicine/Rheumatology/Hands/Hand9/Han
d9.jpeg

Even more AIHA

Alloimmune Hemolytic Anemia
Hemolytic disease of the newborn (HDN)
RhD negative mom developing antibodies to RhD
positive fetal RBCs.
Mild anemia to fatal results
Hydrops fetalis seen in severe HDN
Treatment
RhoGAM with any fetomaternal hemorrhage event or
post-delivery
Intrauterine and intravascular fetal transfusion with
plasma exchange and/or IV IG
http://www.yamagiku.co.jp/pathology/image/219/1.jpg

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

Pallor, jaundice, fatigue, DOE are common in both.

Presentations will overlap, making them difficult to
differentiate.

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

Thrombosis may worsen acutely, leading to renal failure & death

http://dermatlas.med.jhmi.edu/derm/display.cfm?ImageID=1223608648

Hemolytic Uremic
Syndrome

Early childhood (6 mos 4 y/o)

One of the MC causes of ARF
Presentation

ARF
MAHA
http://www.dental.mu.edu/oralpath/lesions/petechia/petechia2.jpeg
Fever
Thrombocytopenia
Clinical - restless, irritable, petechiae, purpura and oozing
may occur

Often follows bacterial/viral illness

Microthrombi localized to kidneys
Treatment
Mild cases fluid & electrolyte correction
Steroids
Plasma exchange

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!

Mustache Day 2005

Quiz answers..
1.

Diagnosis of hemolytic anemia include which of the following?

Increased retic count

2.

Which is the most common lymphoproliferative disorder

associated with warm-type AIHA?
Chronic lymphocytic leukemia

3.

Splenectomized patients should:

1.
Receive pneumococcal vaccinations
2.
Receive meningiococcal vaccinations
B&C

4.

Which of the following is TRUE?

HUS is more specific for renal system than TTP

5.

Which of the following should be avoided in treatment of TTP?

Platelet transfusions

6.

Which of the following is the most sensitive marker for

microangiopathic hemolytic anemia in the setting of HELLP?
Decreased serum haptoglobin level