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DR Medhat MRCP

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Chronic Hemolytic Anemia with acute exacerbations<br />

Long standing 1. Mild Jaundice C/P of anemia recurrent crisis<br />

2. HSM A. Hemolytic crisis<br />

3. Gall stones. - Fever with rigors<br />

4. Reticulocytosis. - Loin pain<br />

5. Indirect hyperbilirubinemia. - Bone aches.<br />

6. Low (consumed) haptoglobin. - pallor &<br />

7. High LDH. jaundice<br />

8. BM erythroid hyperplasia B. Aplastic crisis.<br />

( may interrupted by hypoplasia C. Sickle cell crisis<br />

During infection`aplastic crisis`)<br />

(only in sickle cell disease)<br />

Vascular occlusive crisis<br />

Sequestration crisis.<br />

Transfusion Reactions<br />

• Acute complications(i.e during transfusion to few hours after) :<br />

1. Acute intravascular hemolysis (anti A & B IgM), ≥ 4 mm incompatible ABO is fatal.<br />

2. Acute bacterial infection & shock ( rare in RBCs but common with platelet<br />

transfusion `stored in room temp.`)<br />

3. Transfusion related acute lung injury (TRALI) [donor plasma HLA against patient`s<br />

neutrophils→cytokines→↑ alveolar capillary permeability→non cardiogenic<br />

pulmonary oedema →acute respiratory distress within 6 hrs after transfusion.<br />

4. Fluid overload & pulmonary edema.<br />

5. Non-hemolytic febrile reaction to transfused platelets & RBCs (fever + rigors).<br />

6. Anaphylaxis (type 1 hypersensitivity `IgE mediated`)<br />

• Delayed complications (24 hours after transfusion due to development of RBCs<br />

Alloantibodies → uexplained pyrexia ,jaundice & anemia ):<br />

1. Delayed extravascular hemolysis of transfused RBCs<br />

- IgG mediated , More common than acute intravascular hemolysis.<br />

2. Development of Abs in patient plasma → allo-immunisation.<br />

3. Post transfusion purpura.<br />

4. Iron overload.<br />

5. Chronic infection (Hepatitis , HIV).<br />

6. Immunmodulation.<br />

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