Montgomery Hunter, Luo Matthew X, Baker Steven, Lim Ming Y
University of Utah School of Medicine, Salt Lake City, UT, USA.
Department of Pathology, University of Utah, Salt Lake City, UT, USA.
Case Rep Med. 2023 Dec 31;2023:5290115. doi: 10.1155/2023/5290115. eCollection 2023.
Hyperhemolysis syndrome (HS) is a severe hemolytic transfusion reaction that can cause hemoglobin and hematocrit levels to drop below pretransfusion levels, leading to severe anemia. HS most commonly occurs in patients with a pre-existing hemoglobinopathy such as sickle cell disease (SCD) or beta-thalassemia.
We report a case of HS, occurring in the absence of hemoglobinopathy, making the diagnosis challenging. The patient reported was also affected by a CIC-rearranged sarcoma. As part of the workup, the patient received a bone marrow biopsy for suspected hemophagocytic lymphohistiocytosis.
This provided a rare biopsy specimen to correlate reticulocytopenia with marked erythroid hyperplasia in the marrow, supporting the hypothesis of reticulocyte destruction as a contributing cause of anemia in these patients. This patient had demonstrable alloantibodies to the Jk(a) and P1 antigens as potential triggers for HS.
It is vital that a diagnosis of HS be correctly made in these patients with severe anemia, as blood transfusions generally lead to worsening of their conditions.
高溶血综合征（HS）是一种严重的溶血性输血反应，可导致血红蛋白和血细胞比容水平降至输血前水平以下，引发严重贫血。HS最常发生于患有镰状细胞病（SCD）或β地中海贫血等既往存在血红蛋白病的患者中。
我们报告一例无血红蛋白病情况下发生的HS病例，这使得诊断具有挑战性。报告的患者还患有CIC重排肉瘤。作为检查的一部分，患者因疑似噬血细胞性淋巴组织细胞增生症接受了骨髓活检。
这提供了一份罕见的活检标本，将网织红细胞减少与骨髓中明显的红系增生相关联，支持网织红细胞破坏是这些患者贫血的一个促成原因的假说。该患者体内可检测到针对Jk(a)和P1抗原的同种抗体，这可能是HS的触发因素。
对于这些严重贫血患者，正确诊断HS至关重要，因为输血通常会导致其病情恶化。
