关键词: Haptoglobin LDH Normal Schistocyte Thrombotic microangiopathy

Mesh : Biopsy Erythrocytes, Abnormal Fatal Outcome Hemolysis Hepatomegaly / complications Humans Inflammation Integrin beta3 / metabolism L-Lactate Dehydrogenase / metabolism Liver / pathology Lung / pathology Male Microcirculation Middle Aged Splenomegaly / complications Stem Cell Transplantation Thrombocytopenia / complications Thrombosis / metabolism Thrombotic Microangiopathies / complications therapy Transplantation, Autologous

来  源:   DOI:10.1016/j.amjms.2020.01.019   PDF(Sci-hub)

Abstract:
Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia with thrombocytopenia. In addition to the primary TMA syndromes, microangiopathic hemolytic anemia with thrombocytopenia can be seen in many systemic diseases. Transplant associated TMA (TA-TMA) affects patients following stem cell or solid organ transplant. A 48-year-old male who underwent autologous stem cell transplant for nonsecretory multiple myeloma was admitted to our hospital with worsening anemia, thrombocytopenia, renal dysfunction and hepatosplenomegaly. Initial blood work revealed rare schistocytes and normal lactate dehydrogenase and haptoglobin levels. He underwent an extensive workup looking for an infectious, inflammatory or malignant etiology but a definitive diagnosis could not be reached. Over his prolonged stay at the hospital, he suffered from multiorgan failure and eventually passed away. An autopsy revealed TMA involving all clinically affected organ systems and was deemed to be the cause of his demise. The absence of typical blood work suggestive of hemolysis does not rule out a diagnosis of TA-TMA. Knowledge of this rare disease entity will help physicians identify and treat this life-threatening condition early and effectively.
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