Refractory Immune Thrombocytopenic Purpura With Low Immature Platelet Fraction

难治性免疫性血小板减少性紫癜伴低未成熟血小板比例

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Abstract

Diagnosis of immune thrombocytopenic purpura (ITP) remains challenging due to the lack of a "gold standard test," with current approaches relying on clinical evaluation, complete blood count, and peripheral blood smear. The immature platelet fraction (IPF) has emerged as a useful tool for differentiating ITP from hypoproliferative thrombocytopenia, typically presenting as elevated in ITP cases. IPF measures the percentage of immature platelets and is primarily used to help differentiate between thrombocytopenia caused by decreased production, such as bone marrow disorders, and destruction, like ITP.  We present a unique case of a 43-year-old woman with severe thrombocytopenia, initially presenting with a platelet count of zero and a low IPF. Despite multiple platelet transfusions and high-dose dexamethasone, her platelet count remained critically low. Bone marrow biopsy findings confirmed ITP, leading to a revised treatment strategy that included intravenous immunoglobulin (IVIG) and rituximab, resulting in sustained platelet recovery. This case underscores the diagnostic and therapeutic challenges associated with ITP, particularly in patients with atypical presentations. The presence of a low IPF complicated initial diagnostic considerations, necessitating a bone marrow biopsy to exclude alternative etiologies such as bone marrow failure. Additionally, patient-specific factors, including diabetes mellitus, chronic pancreatitis, and prior alcohol use disorder, may have contributed to the severity of thrombocytopenia. Our findings highlight the need for individualized diagnostic approaches and tailored treatment strategies in severe and refractory ITP cases.

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