Tuberculosis reactivation related with ruxolitinib in a patient with primary myelofibrosis

Primary myelofibrosis (PMF) is a clonal stein cell disease, characterized by bone marrow fibrosis. Ruxolitinib is a selective inhibitor of JAK-1 and JAK-2 used to treat PMF. Its mechanism of action is based on the reduction of signal transduction and cytokine levels; including IL-6 and tumor necrosis factor alpha. Increased infection risk related to Ruxolutinib is rarely reported. Here we describe a case of tuberculosis infection ractivation in a female patient treated with Ruxolitinib. During the treatment, she complained of night sweats, weight loss and enlarged mass in the neck. Excisional mass biopsy revealed a necrotizing granulomatous lymphadenitis. QuantiFERON-TB and PPD tests were not able to diagnose the tuberculosis infection. Therapy with Ruxolitinib was interrupted due to possible immunsuppressive effects and the patient was treated with the standard antituberculosis regimen. After six months, the patient's symptoms had resolved and there was no lymphoadenopathy. In conclusion, it is important to assess the risk of tuberculosis activation before Ruxolitinib treatment. In addition, the diagnosis of tuberculosis using QuantiFERON-TB and PPD may be misleading in patients treated with Ruxolutinib

Assessment of Endothelial Dysfunction With Flow-Mediated Dilatation in Myeloproliferative Disorders.

Thrombosis is the most important cardiovascular complication of classical myeloproliferative disorders (MPDs). Endothelial dysfunction (ED) is known to play a major role in the mechanism of thrombophilia in MPDs

Assessment of Endothelial Dysfunction With Flow-Mediated Dilatation in Myeloproliferative Disorders

Background: Thrombosis is the most important cardiovascular complication of classical myeloproliferative disorders (MPDs). Endothelial dysfunction (ED) is known to play a major role in the mechanism of thrombophilia in MPDs. Methods: Endothelial dysfunction and its associations with other parameters were investigated. A total of 18 patients with polycythemia vera (PV), 24 with essential thrombocytosis (ET), 7 with primary myelofibrosis (PMF), and 30 healthy patients as a control group were included in the study. To assess the ED, flow-mediated dilatation (FMD) measurements were used. Results: The FMD (\%) result showing ED was determined as 9.9 (0.0-21.6) in the patients with PV, 7.3 (0.0-30.5) in patients with ET, 7.5 (0.0-18.0) in patients with PMF, and 13.9 (6.2-26.7) in the control group. The FMD (\%) was markedly impaired in all patients with MPD compared to the control patients (7.8 {[}0.0-30.5] vs 13.9 {[}6.15-26.8], P = .02). According to the disease subtypes, FMD (\%) was significantly lower in the ET group than in the control group (P = .01). Conclusion: Endothelial function was assessed in patients with MPD having FMD and was determined to demonstrate ED. Lower FMD was associated with older age, leukocytosis, thrombocytosis, and thrombosis history

A proven case of cutaneous rhizopus infection presenting with severe limb pain very soon after induction treatment in a patient with acute lymphoblastic leukemia.

Objective and Importance. Invasive mucormycosis may complicate the course of patients with hematologic malignancies and has a very high mortality rate. Early diagnosis and aggressive approach combined with surgical and medical treatment have paramount importance for cure. Clinical Presentation. We report here a case of a patient with acute lymphoblastic leukemia presenting with a subcutaneous mass lesion which was sampled by an ultrasound guided needle biopsy. The pathology showed microorganisms with aseptate hyphae with wide, irregular walls and more or less branching with highly vertical angles which suggested a mold infection. The specimen was also cultured where Rhizopus spp. grew. Conclusion. Posaconazole 200 mg QID was commenced. She recovered from neutropenia and pain on day 20 of treatment. After 4 courses of hyper-CVAD chemotherapy, the remaining soft tissue mass was removed surgically and she underwent allogeneic HSCT from a full matched sibling donor under secondary prophylaxis

Secondary antifungal prophylaxis in allogeneic hematopoietic stem cell transplant recipients with invasive fungal infection

Introduction: Invasive fungal infection (IFI) is a major cause of morbidity and mortality in allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients. A previous history of IFI is not an absolute contraindication for allo-HSCT, particularly in the era of secondary antifungal prophylaxis (SAP). Prompt diagnosis and therapy are essential for HSCT outcome. Methodology: The charts of 58 allo-HSCT recipients. median age:29.5 (16-62); M/F:41/17. who had a previous history of IFI were retrospectively reviewed. Results: Possible IFI was demonstrated in 32 (55.2\%), probable in 13 (22.4\%) and proven in 13 patients (22.4\%). All patients received SAP. liposomal amphoterisin B (n = 35), voriconazole (n = 17), caspofungin (n = 2), posaconazole (n = 1), combination therapy (n = 3). which was started on the first day of the conditioning regimen. Treatment success was better in the voriconazole group when compared to the amphotericin B arm (100\% vs 69.2\%; p = 0.029). Development of breakthrough IFI was more frequent in patients on amphotericin B prophylaxis (42.4\% vs 23.1\%; p = 0.036). Clinical and radiological response were achieved in 13 of 18 patients (72.2\%) who developed breakthrough infection. Overall survival of the study population was 13.5\% at a median follow-up of 154 (7-3285) days. Fungal mortality was found to be 23\%. Overall survival was better in the voriconazole arm, without statistical significance (90\% vs 65.8\%, p > 0.05). Conclusions: Secondary antifungal prophylaxis is considered to be an indispensible strategy in patients with pre-HSCT IFI history. Voriconazole seems to be a relatively better alternative despite an underlying necessity of larger prospective trials