Abstract
The differential diagnosis of immune (ITP) and hereditary macrothrombocytopenia (HM) is key to patient management. The immature platelet fraction (IPF) represents the subset of circulating platelets with higher RNA content, and has been shown to distinguish hypo- from hyperproliferative thrombocytopenias. Here we evaluated the diagnostic accuracy of IPF in the differential diagnosis between HM and other thrombocytopenias in a population of patients with post-chemotherapy thrombocytopenia (n = 56), bone marrow failure (n = 22), ITP (n = 105) and HM (n = 27). TPO levels were also measured in HM and ITP matched for platelet counts. Platelet counts were similar in all patient groups. Higher IPF values were observed in both ITP (12.3%; 2.4–65.6%) and HM (29.8%; 4.6–65.9%) compared to hypoproliferative thrombocytopenias. IPF values were also higher in HM compared to ITP, yielding a diagnostic accuracy of 0.80 (95%CI 0.70–0.90; P < 0.0001) to distinguish these two conditions. Intra- and inter-assays reproducibility of IPF in HM patients revealed that this is a stable parameter. In conclusion, IPF is increased in HM compared to both ITP and other thrombocytopenias and contributes to the differentiation between ITP and HM. Further studies are warranted to understand the biological rationale of these findings and to its incorporation in diagnostic algorithms of HM.
Highlights
The differential diagnosis of thrombocytopenias includes a variety of conditions such as hematologic malignancies, bone marrow failure (BMF), hypersplenism, immune thrombocytopenia (ITP), microangiopathic hemolytic anemias and hereditary macrothrombocytopenia (HM)
The development of hematological analyzers in the last decades has allowed the incorporation of new parameters in the diagnosis of hematological disorders; and the feasibility of this strategy for the diagnosis of HM has been shown by a series of studies evaluating platelet size in these patients
These studies demonstrated that mean platelet volume (MPV) is significantly higher in patients with HM compared to ITP4, a finding that was later validated in a multicenter study using different instruments to measure this parameter[5]
Summary
The differential diagnosis of thrombocytopenias includes a variety of conditions such as hematologic malignancies, bone marrow failure (BMF), hypersplenism, immune thrombocytopenia (ITP), microangiopathic hemolytic anemias and hereditary macrothrombocytopenia (HM). The feasibility of using parameters of the complete blood count (CBC) to support this differential diagnosis was illustrated by a series of studies which demonstrated and validated that the mean platelet volume (MPV) can help the segregation of patients with ITP and HM4, 5. Studies have reported the clinical utility of measuring immature platelets in clinical settings using automated hematology analyzers[8,9,10]. In 2000, Fabris et al evaluated reticulated platelets by flow cytometry in a population of 29 patients with HM, observing reduced IPF values when compared to ITP13. To further elucidate the value of IPF determination in the diagnosis of thrombocytopenias, with focus on the differential diagnosis between ITP and HM, we investigated the diagnostic accuracy and the precision of IPF measurements in a population of patients with different causes of thrombocytopenia, including a well-characterized cohort of patients with HM. We evaluated whether thrombopoietin (TPO) levels could further facilitate this diagnosis
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