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Cold Agglutinin Disease: Pathogenesis

Cold agglutinin disease is a rare form of autoimmune hemolytic anemia caused by monoclonal IgM autoantibodies that agglutinate red blood cells at low temperatures. These cold agglutinins activate the classical complement pathway, leading to chronic red blood cell destruction or hemolysis. Patients present with mild anemia, fatigue, weakness, and other symptoms. Diagnosis involves testing blood samples at 37°C to identify cold agglutinin activity and antibody specificity. Treatment depends on disease severity, with supportive care for acute cases and rituximab-fludarabine or plasmapheresis for chronic cases.

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Michelle San Miguel Feguro
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145 views2 pages

Cold Agglutinin Disease: Pathogenesis

Cold agglutinin disease is a rare form of autoimmune hemolytic anemia caused by monoclonal IgM autoantibodies that agglutinate red blood cells at low temperatures. These cold agglutinins activate the classical complement pathway, leading to chronic red blood cell destruction or hemolysis. Patients present with mild anemia, fatigue, weakness, and other symptoms. Diagnosis involves testing blood samples at 37°C to identify cold agglutinin activity and antibody specificity. Treatment depends on disease severity, with supportive care for acute cases and rituximab-fludarabine or plasmapheresis for chronic cases.

Uploaded by

Michelle San Miguel Feguro
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd

Cold Agglutinin Disease

- RARE FORM OF autoimmune hemolytic anemia in hepatic macrophages, which have receptors
which the body mistakenly recognizes the antigen for C3b.
on the surface on RBC as foreign this due the  However, if the autoantibody has a high
production of cold agglutinin CA autoantibodies thermal amplitude or there is a deficiency in
that activate the classical complement pathway complement regulatory proteins, full
leading to chronic rbc destruction or hemolysis this complement activation and intravascular
ca are usually igm hemolysis can occur.

 Autoantibodies of the IgM class that react EPIDEMIOLOGY


optimally at 4° C and are commonly found in  CAD has recently been recognized as a clonal
healthy individuals. lymphoprolifera-tive B cell disorder.
 It comprises approximately 15% to 20% of
 nonpathologic cold agglutinins are the cases of autoimmune hemolytic anemia.
polyclonal, occur in low titers (less than  In a study of 86 patients with chronic CAD by
1:64 at 4° C), and have no reactivity above Berentsen and colleagues, the median age at
30° C. onset was 67 years, with the median age at
 pathologic cold agglutinins are monoclonal, death reported to be 82 years.
occur at high titers (greater than 1:1000 at
4° C), and are capable of reacting at Clinical manifestations in chronic CAD
temperatures greater than 30° C.
 mild anemia
ETIOLOGY  fluctuations
Acute CAD  Symptoms include:
 POLYCLONAL IGM  Fatigue
 K AND I LIGHT CHAINS  Weakness
 Dyspnea
SECONDARY  pallor
 Mycoplasma pneumoniae infection  Acrocyanosis
 infectious mononucleosis  Hemoglobinuria
 other viral infections. CLINICAL SIGNIFICANCE IN ACUTE CAD
Chronic CAD  have mild to severe hemolysis
 MONOCLONAL IGM  other viral infection, or M. pneumoniae
 K LIGHT CHAINS infection
 idiopathic
BLOOD SPECIMENS
SECONDARY  must be maintained at 37° C for 15 minutes
lymphoproliferative neoplasms  Alternatively, anticoagulated with EDTA can be
warmed for 15 minutes
PATHOGENESIS  slide can also be warmed to 37° C prior to the
 In CAD, the IgM autoantibody binds to RBCs application of blood.
after exposure to the cold, particularly in the
peripheral circulation and the vessels of the RBC agglutination grossly
skin, where temperatures can drop to 30°C.  elevates the mean cell volume
 During the brief transit through these colder  reduces the RBC count
areas, IgM auto-antibodies activate the  unpredictable effects on other indices
classical complement pathway.  interfere with ABO typing
 When the RBCs return to the central
circulation, the IgM antibody dissociates, but
C3b components remain on the cell.
 Hemolysis is predominantly extravascular by
LABORATORY TESTING
 DAT or Direct antiglobulin test - positive with
polyspecific AHG
 The specificity :anti-I or anti-i or, anti-Pr
(rare)
 A cold agglutinin method is used to determine
the titer of the antibody at 4° C.
 The agglutination can also be observed on a
peripheral blood film

TREATMENT
- ACUTE CAD
 no treatment is required
 transfusion and supportive care.
-CHRONIC CAD
 regularly monitored
 advised to avoid cold temperatures
 Rituximab-fludarapine
 Plasmapheresis

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