Acquired Hemolytic Anemia (222)

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Acquired Hemolytic Anemia (222)

• Eric Quimbo, DO
• June 8, 2006

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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

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Presentation

• Weakness
• Dizziness
• DOE
• Tachycardia
• Murmur
• Abdominal pain
• Pallor
• Jaundice

• Fever, lymphadenopathy
• systemic disease
• Splenomegaly
• Chronic
• Hepatomegaly
• Darkened urine
• Intravascular hemolysis, hemoglobinuria

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Diagnosis

• Spherocytes/schistocytes on peripheral smear
• ?Hct
• ?Retic count
• ?indirect bilirubin (? Hgb breakdown)
• ?LDH

http//www.wadsworth.org/chemheme/heme/glass/cytop
ix/slide014schisto2.jpg
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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

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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

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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 1-3 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

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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/Rh
eumatology/Hands/Hand9/Hand9.jpeg
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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.jp
g
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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
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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.
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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 lt 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

Neurologic impairment
http//dermatlas.med.jhmi.edu/derm/display.cfm?Ima
geID1223608648
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Hemolytic Uremic Syndrome

• Early childhood (6 mos 4 y/o)
• One of the MC causes of ARF
• Presentation
• ARF
• MAHA
• 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

http//www.dental.mu.edu/oralpath/lesions/petechia
/petechia2.jpeg
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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

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THE END!
Mustache Day 2005
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Quiz answers..

• Diagnosis of hemolytic anemia include which of
  the following?
• Increased retic count
• Which is the most common lymphoproliferative
  disorder associated with warm-type AIHA?
• Chronic lymphocytic leukemia
• Splenectomized patients should
• Receive pneumococcal vaccinations
• Receive meningiococcal vaccinations
• B C
• Which of the following is TRUE?
• HUS is more specific for renal system than TTP
• Which of the following should be avoided in
  treatment of TTP?
• Platelet transfusions
• Which of the following is the most sensitive
  marker for microangiopathic hemolytic anemia in
  the setting of HELLP?
• Decreased serum haptoglobin level