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Bharat Kumar Peddinani

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Internal Medicine

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Internal Medicine

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Internal Medicine

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Internal Medicine

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Internal Medicine

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Patient Care

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Research/Clinical

Abstract

Introduction: Delayed hemolytic transfusion reaction (DHTR) in adults with sickle cell disease (SCD) is frequently mistaken for vaso-occlusive crisis (VOC) or evolving acute chest syndrome (ACS). New alloantibodies may be absent or delayed, so reflex transfusion can worsen hemolysis. Programs now emphasize routine post-transfusion surveillance and a protocolized, transfusion-sparing response when DHTR is suspected.

Case Presentation: A 35-year-old man with hemoglobin SS SCD, hypertension, chronic kidney disease, and recurrent priapism presented with severe left-leg pain two weeks after receiving two units of packed red blood cells for pre-operative optimization. Baseline hemoglobin (Hb) was ~6 g/dL; he was off disease-modifying therapy. He was afebrile and stable. Initial studies showed Hb 6.3 g/dL, lactate dehydrogenase (LDH) 666 U/L, total bilirubin 3.6 mg/dL (direct 0.39), creatinine 2.57 mg/dL, and white blood cells 21.8×10⁹/L, with an unremarkable chest radiograph and electrocardiogram. VOC was presumed and he received intravenous hydration, hydromorphone, ceftriaxone, folic acid, and hydroxyurea.

Over 48 hours, hemolysis accelerated: Hb fell to 5.2 g/dL (days 3–4), LDH rose to 5,359 U/L, indirect bilirubin increased to 6.5 mg/dL, and creatinine peaked at 3.60 mg/dL with new jaundice. The direct antiglobulin test was positive for IgG and C3 with warm autoantibodies. Given the two-week interval after transfusion and a Hb decline below the pre-transfusion level, DHTR with features of hyperhemolysis was diagnosed. Further transfusion was withheld. High-dose immunomodulation was initiated (methylprednisolone 125 mg IV q12h plus intravenous immunoglobulin 1 g/kg daily ×2), alongside supportive care and hydroxyurea. Within 72 hours, LDH fell to 3,235 U/L, indirect bilirubin to 4.4 mg/dL, Hb stabilized at 5.4 g/dL without additional transfusion, creatinine improved to 3.12 mg/dL, and symptoms abated.

Discussion: This course illustrates a recognized diagnostic pitfall: a post-transfusion SCD admission that initially resembles routine VOC but diverges with a falling Hb, rising hemolysis markers, and jaundice. Adult series show that DHTR clusters in intermittently transfused patients and that serology may be negative or delayed; only a subset of hyperhemolysis episodes are direct antiglobulin test (DAT)–positive with complement detected. Accordingly, diagnosis should be guided by the trajectory—recent transfusion, Hb dropping below the pre-transfusion baseline, biochemical hemolysis, and often reticulocytopenia—with hemoglobin A percentage (HbA%), when available, used to heighten sensitivity and distinguish DHTR from other causes of anemia. Contemporary practice favors rapid coordination with transfusion medicine, transfusion avoidance unless anemia is life-threatening, early corticosteroids and intravenous immunoglobulin, and targeted escalation (e.g., complement blockade) for refractory hyperhemolysis, coupled with prevention strategies such as extended antigen matching and RH genotyping.

Conclusion: DHTR should remain high on the differential in any post-transfusion SCD admission, including cases that present as VOC. Early identification—anchored in daily Hb trends and hemolysis markers and supported by HbA% when available—enables timely, transfusion-sparing immunomodulation and coordinated care that limits iatrogenic harm.

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When a Pain Crisis Isn’t only a Crisis: Delayed Hemolytic Transfusion Reaction with Hyperhemolysis in Adult Sickle Cell Disease.

Introduction: Delayed hemolytic transfusion reaction (DHTR) in adults with sickle cell disease (SCD) is frequently mistaken for vaso-occlusive crisis (VOC) or evolving acute chest syndrome (ACS). New alloantibodies may be absent or delayed, so reflex transfusion can worsen hemolysis. Programs now emphasize routine post-transfusion surveillance and a protocolized, transfusion-sparing response when DHTR is suspected.

Case Presentation: A 35-year-old man with hemoglobin SS SCD, hypertension, chronic kidney disease, and recurrent priapism presented with severe left-leg pain two weeks after receiving two units of packed red blood cells for pre-operative optimization. Baseline hemoglobin (Hb) was ~6 g/dL; he was off disease-modifying therapy. He was afebrile and stable. Initial studies showed Hb 6.3 g/dL, lactate dehydrogenase (LDH) 666 U/L, total bilirubin 3.6 mg/dL (direct 0.39), creatinine 2.57 mg/dL, and white blood cells 21.8×10⁹/L, with an unremarkable chest radiograph and electrocardiogram. VOC was presumed and he received intravenous hydration, hydromorphone, ceftriaxone, folic acid, and hydroxyurea.

Over 48 hours, hemolysis accelerated: Hb fell to 5.2 g/dL (days 3–4), LDH rose to 5,359 U/L, indirect bilirubin increased to 6.5 mg/dL, and creatinine peaked at 3.60 mg/dL with new jaundice. The direct antiglobulin test was positive for IgG and C3 with warm autoantibodies. Given the two-week interval after transfusion and a Hb decline below the pre-transfusion level, DHTR with features of hyperhemolysis was diagnosed. Further transfusion was withheld. High-dose immunomodulation was initiated (methylprednisolone 125 mg IV q12h plus intravenous immunoglobulin 1 g/kg daily ×2), alongside supportive care and hydroxyurea. Within 72 hours, LDH fell to 3,235 U/L, indirect bilirubin to 4.4 mg/dL, Hb stabilized at 5.4 g/dL without additional transfusion, creatinine improved to 3.12 mg/dL, and symptoms abated.

Discussion: This course illustrates a recognized diagnostic pitfall: a post-transfusion SCD admission that initially resembles routine VOC but diverges with a falling Hb, rising hemolysis markers, and jaundice. Adult series show that DHTR clusters in intermittently transfused patients and that serology may be negative or delayed; only a subset of hyperhemolysis episodes are direct antiglobulin test (DAT)–positive with complement detected. Accordingly, diagnosis should be guided by the trajectory—recent transfusion, Hb dropping below the pre-transfusion baseline, biochemical hemolysis, and often reticulocytopenia—with hemoglobin A percentage (HbA%), when available, used to heighten sensitivity and distinguish DHTR from other causes of anemia. Contemporary practice favors rapid coordination with transfusion medicine, transfusion avoidance unless anemia is life-threatening, early corticosteroids and intravenous immunoglobulin, and targeted escalation (e.g., complement blockade) for refractory hyperhemolysis, coupled with prevention strategies such as extended antigen matching and RH genotyping.

Conclusion: DHTR should remain high on the differential in any post-transfusion SCD admission, including cases that present as VOC. Early identification—anchored in daily Hb trends and hemolysis markers and supported by HbA% when available—enables timely, transfusion-sparing immunomodulation and coordinated care that limits iatrogenic harm.

 

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