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Hematology Chapter 17
Hemolytic Anemia: Immune Anemias
32
Biology
Graduate
12/13/2012

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Term
antibody systems cold agglutinin syndrome
Definition
IgM and complement activation due to reaction against I/i antigens
Term
antibody systems paroxysmal cold hemoglobinuria
Definition
Donath Landsteiner antibody which is a biphastic IgG, that fixes complement, reactive to autoanti-P antibody
Term
antibody systems warm autoimmune hemolytic anemias
Definition
IgG that reacts with rH protein complex
Term
DAT
Definition
direct AHG (anti-human globulin) test which detects RBC coated in IgG in vitro; it is used to differentiate autoimmune hemolytic anemias from other hemolytic anemias
Term
DAT in cold agglutinin syndrome
Definition
monoclonal IgM (kappa) attached to RBCs due to autoanti I specificity
Term
DAT in warm autoimmune hemolytic anemia
Definition
positive for IgG in serum for autoantibodies, positive for complement, polyspecific
Term
hematological findings cold agglutinin syndrome
Definition
Chronic or episodic hemolytic anemia, Agglutination (in cold areas of body), Vascular changes (Acrocyanosis- blue hands/feet and Raynaud’s phenomenon- pain with skin color changes due to blood vessel spasm), Hemoglobinuria, Blood counts (Falsely ↓ RBC and Falsely ↑ MCV due to clumping), Mild to moderate anemia. Normocytic/normochromic. Polychromasia, spherocytes, clumps of RBCs, NRBCs, erythrophagocytosis, Bone marrow (Normoblastic hyperplasia) and Splenomegaly
Term
hematological findings warm autoimmune hemolytic anemia
Definition
can occur at any age (usually over 40), normal anemia symptoms, secondary to underlying disorder, Immune mediated hemolysis (autoantibodies in serum, spherocytes), peripheral blood (normocytic/normochromic anemia, retics, polychrome, nRBCs, spherocytes, schistocytes, erythrophagocytosis)
Term
hematological findings HDFN: ABO type
Definition
NRBC, schistocytes, spherocytes, Polychromasia in babies blood, anti-A &/or anti-B in moms blood, mild anemia, moderate bilirubinemia (in baby), DAT weakly positive or negative
Term
hematological findings HDFN: Rh type
Definition
Marked Polychromasia, mild or absent poikilocytosis, few (if any) spherocytes in baby, moderate to severe anemia and bilirubinemia. DAT positive, no spherocytes, caused by anti-D in mom
Term
Alloimmune stimulus for antibody production
Definition
Antigens on transfused cells are lacking on RBCs of recipient, causes production of alloab that react with cells that have the foreign Ag
Term
Alloimmune site of hemolysis
Definition
can be intravascular (IgM, immediate) or extravascular (IgG, delayed)
Term
Alloimmune type of antibody involved
Definition
IgM or IgG
Term
Alloimmune DAT
Definition
positive (transient positive acute transfusion reaction, weakly positive/negative in HDFN ABO type)
Term
Alloimmune IAT
Definition
positive, indicative of alloimmune, ABO HDFN screened via IAT
Term
Autoimmune stimulus for antibody production
Definition
immune reaction against self-antigens
Term
Autoimmune site of hemolysis
Definition
extravascular (RBC sensitized with Ab or complement) or intravascular (MAC initiated)
Term
Autoimmune type of antibody involved
Definition
IgG (warm) or IgM (cold)
Term
Autoimmune DAT
Definition
positive
Term
Autoimmune IAT
Definition
positive for cold agglutination
Term
immediate hemolytic tranfusion reactions
Definition
IgM
Term
delayed hemolytic transfusion reactions
Definition
IgG
Term
Extravascular hemolysis site of destruction
Definition
spleen or liver
Term
Extravascular hemolysis immunoglobulin class of antibody
Definition
IgM (more effective) or IgG
Term
Extravascular hemolysis underlying mechanism
Definition
Ab Sensitized cells phagocytized by macrophages in spleen or liver
Term
Intravascular hemolysis site of destruction
Definition
blood vessels
Term
Intravascular hemolysis immunoglobulin class of antibody
Definition
IgM
Term
Intravascular hemolysis underlying mechanism
Definition
Complement cascade activated → C9 (MAC) → RBC lysis
Term
PCH hemolysis
Definition
intravascular, MAC initiated due to biphasic autoantibodies to P antigen on RBC
Term
PCH confirmatory test
Definition
test for D-L (Donath Landsteiner) antibodies
Term
Prenatal pathophysiology
Definition
Mother forms alloantibodies against fetal RBCs antigens, IgG antibodies cross placenta and destroy fetal RBC in utero
Term
Postnatal pathophysiology
Definition
anemia and bilirubinemia
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