Successful preventive treatment with cyclosporine in a patient with relapsed/refractory immune-mediated thrombotic thrombocytopenic purpura: a case report and review of the literature.

Although salvage therapy with rituximab is effective in some cases of immune-mediated thrombotic thrombocytopenic purpura (iTTP) refractory to standard plasma exchange (PEX) and glucocorticoid treatment or relapsed after treatment, protocols to address the subsequent high recurrence rate have not been established. We describe the use of cyclosporine (CSA) to prevent recurrence in a patient with iTTP relapse after rituximab therapy, and present a literature review. A 24-year-old woman was diagnosed with iTTP and initially received PEX and high-dose methylprednisolone therapy. However, weekly rituximab therapy was also needed for inhibitor boosting to achieve additional immunosuppression during the initial treatment. Although the patient achieved clinical remission after weekly rituximab therapy, iTTP relapsed twice when glucocorticoids were tapered, and was treated with a triplet regimen consisting of PEX, high-dose methylprednisolone, and weekly rituximab. CSA was administered along with glucocorticoids as prophylaxis against iTTP relapse. The additional CSA therapy successfully maintained iTTP remission and allowed reduction of the corticosteroid dose. Our findings demonstrate that prophylactic CSA can potentially prevent iTTP recurrence in patients with a history of multiple relapses. Data from more cases must be accumulated to establish a useful prophylactic therapy for iTTP that is refractory even to rituximab.

Cytopenia associated with copper deficiency.

Introduction: Due to an increased incidence of copper deficiency, we investigated adult patients who had low serum levels of copper with cytopenia at our hospital from March 2014 to March 2021. Methods: We retrospectively reviewed the clinical data of patients who had been diagnosed with cytopenia due to copper deficiency at the Aichi Medical University Hospital from March 2014 to March 2021. Results: In the 15 patients with cytopenia secondary to low serum copper level, 11 had cytopenia of two to three lineages; three (27%) had pancytopenia, and eight (73%) had bicytopenia. Of the 15 patients, nine (60%) underwent bone marrow examinations; three (30%) showed typical morphologic features associated with copper deficiency, such as multiple clear cytoplasmic vacuoles in erythroblasts and myeloid cells, and three (30%) showed dysplastic features as observed in myelodysplastic syndrome. Among the 14 (93%) patients who were treated with copper supplements, had cessation of zinc supplements, or both, 11 (73%) and eight (53%) showed normal copper levels and hematological improvement, respectively. Conclusion: Copper deficiency is more common than expected and should be considered in patients with unexplained cytopenia.

Pretreatment Immature Platelet Fraction as a Surrogate of Reticulated Platelets Predicts the Response to Corticosteroids in Adults with Immune Thrombocytopenia.

OBJECTIVES: Reticulated platelets circulating in the blood reflect megakaryopoietic activity and platelet turnover and can be automatically and low-invasively measured as the immature platelet fraction (IPF) using a Sysmex XN hematocytometer. The present study retrospectively investigated whether or not the IPF can predict the treatment response to corticosteroids in adult patients with primary immune thrombocytopenia (ITP). METHODS: Forty-six patients who had been newly diagnosed with primary treatment-naïve ITP and started treatment with corticosteroids were analyzed. RESULTS: Among the 46 primary ITP patients, 33 (72%) responded to the treatment and 13 (28%) did not. The percentage of IPF (IPF%) among the nonresponders was significantly lower than that of the responders (6.6 vs. 16.0%; p < 0.001). In the receiver operating characteristics analysis, the optimum IPF% cut-off value for predicting the treatment response was 12%, with a specificity of 85% and a sensitivity of 76%. CONCLUSIONS: Our findings thus suggest that measuring the IPF% as a surrogate of reticulated platelets is useful to identify patients likely to respond to corticosteroids.

[Emergence of Howell-Jolly bodies in a patient with splenic hypoplasia complicated by fulminant pneumococcal infection].

We report the case of a patient with fulminant pneumococcal infection along with the presence of Howell-Jolly bodies (HJBs) and splenic hypoplasia at the onset. A 71-year-old man developed fever during outpatient chemotherapy for IgG-κ multiple myeloma and was diagnosed with septic shock due to invasive pneumococcal infection. HJBs were observed on peripheral blood smears at this visit. Computed tomography revealed marked hypoplasia of spleen, suggesting the presence of hyposplenic function. Antibacterial therapy was initiated and the pneumococcal infection was cured; however, there was no notable change in his splenic hypoplasia. Splenic hypoplasia can be associated with fatal infections; hence, care should be taken when it is found in the elderly and in patients with cancer and those receiving immunosuppressive treatment. Even today, when automated hematology analyzers have become common, not all patients with hematological diseases have peripheral blood smears checked with a normal optical microscope. This study suggests that HJBs may be useful for simple and rapid screening of splenic hypofunction. The importance of detecting HJBs in peripheral blood smears with a normal optical microscope should be re-recognized.

Favorable prognostic phenotype in myelodysplastic syndrome with der(1;7)(q10;p10).

Unbalanced translocation der(1;7)(q10;p10) is a characteristic chromosomal abnormality in myelodysplastic syndrome (MDS). The current study revealed that among 13 MDS patients with der(1;7)(q10;p10), seven cases with no apparent dysplasia also had low numbers of myeloblasts in the bone marrow and a 3-year survival rate of 86%; in contrast, the remaining six cases had a 3-year survival rate of 0% (P = .003). It was therefore suggested that MDS patients with der(1;7)(q10;p10) are classified into a distinct group with a favorable prognosis and another distinct group with a very poor prognosis.