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Protocols - Hemorio

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SELFIMMUNE HEMOLYTIC ANEMIA<br />

CONCEPT: Selfimmune hemolytic anemia is defined as pathologies in which occurs premature<br />

destruction of red blood cells, mediated by fixes antibodies and antigens of erythrocyte membrane. This<br />

immune fixation triggers a number of reactions in cascade that finishes in these cells lysis<br />

(INTRAVASCULAR HEMOLYSE), in addition to phagocytose by macrophagolitic system<br />

(ETRAVASCULAR HEMOLYSE)<br />

ETHIOLOGIC CLASSICATION<br />

PRIMARY IDIOPATIC<br />

SECONDARY Lynphoproliferative<br />

disease<br />

LLC, DH, LNH, MM<br />

Self-immune disease LES, self-immune hepatitis, rheumatoid<br />

AHAI BY WARM<br />

arthritis, self-immune thyroiditis, Biermer<br />

ANTIBODY<br />

anemia<br />

IgG, C3b<br />

Tumor Ovary Cyst, malignant neoplasm<br />

Infections Pneumonias and rhinopharyngitis<br />

Immunosuppressive Pregnancy and transplants<br />

Drugs Alpha-metildope, cimetidine, Procainamide<br />

AHAI BY COLD<br />

PRIMARY / IDIOPATIC<br />

ANTIBODY (COLD<br />

AGGLUTININ)<br />

SECONDARY<br />

Lynphoproliferative disease<br />

Infections<br />

Cascade complement formation Intravascular hemolyze<br />

Drug absorption in cellular membrane Extra-vascular hemolyze<br />

DRUG-INDUCED AHAI Changes in cellular membrane<br />

Adsorption non-immunologic<br />

PAROXYSM COLD<br />

HEMOGLOBINURIA<br />

(biphasic hemolysins)<br />

Self-antibodies formation Alpha-metildope<br />

Fixes complement at low temperatures and<br />

promotes intravascular hemolyse > 37o IDIOPATIC<br />

C<br />

Diagnose by Donath-Landsteiner test<br />

SECONDA<br />

RY<br />

More frequent in children<br />

associated to viroses<br />

(measles, chickenpox,<br />

rubeola, mononucleosis)<br />

More rare form of AHAI.<br />

DIAGNOSE<br />

Clinical Diagnose: clinical signals of anemia, jaundice. In case of extravascular hemolyze, it may occurs<br />

splenomegaly and in case of cold antibodies, Raynaud phenomena and intolerance to cold, due to vase<br />

spasms, can be noted.<br />

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