B-cell prolymphocytic leukemia (B-PLL) is a rare lymphoid neoplasm with an aggressive clinical course. Treatment strategies for B-PLL remain to be established, and, until recently, alemtuzumab was the only effective therapeutic option in patients harboring 17p deletions. Herein, we describe, for the first time, a case of B-cell prolymphocytic leukemia harboring a 17p deletion in a 48-year-old man that was successfully treated sequentially with idelalisib-rituximab/ibrutinib followed by allogeneic hematopoietic stem cell transplant (allo-HSCT). After 5 months of therapy with idelalisib-rituximab, clinical remission was achieved, but the development of severe diarrhea led to its discontinuation. Subsequently, the patient was treated for 2 months with ibrutinib and the quality of the response was maintained with no severe adverse effects reported. A reduced-intensity conditioning allo-HSCT from a HLA-matched unrelated donor was performed, and, thereafter, the patient has been in complete remission for 10 months now. In conclusion, given the poor prognosis of B-PLL and the lack of effective treatment modalities, the findings here suggest that both ibrutinib and idelalisib should be considered as upfront therapy of B-PLL and as a bridge to allo-HSCT.
Mucormycosis is a rare, highly aggressive and usually fatal infection, which affects immunocompromised patients. This case report describes a patient with acute promyelocytic leukemia who received antifungal therapy for a suspected pulmonary Aspergillus infection. Material from a lobectomy suggested that on histologic grounds the diagnosis had to be changed to mucormycosis. High suspicion of a Mucor infection favors early detection and timely appropriate antifungal therapy, which is crucial for the prognosis of these patients.
CELIAC DISEASE, AUTOIMMUNE ATROPHIC GASTRITIS AND HELICOBACTER PYLORI IN ORAL IRON THERAPY RESISTANCE ANEMIA Objectives: Identify the number of celiac disease (CD), autoimmune atrophic gastritis (AAG) and the Helicobacter pylori (Hp) infection in patients with iron-refractory iron deficiency anemia (RIA). Type of study: Observational, retrospective, longitudinal. Location: Hospital center hematology unit in the north region of Portugal. Population: All first hematology consultations of patients with RIA between 2011 and 2016. Methods: Collection of data using the electronic clinical file (socio-demographic, origin of the consultation request and diagnostic tests data). Microsoft Excel 2016® was used to analyze and process the data. Results: We identified 48 cases of iron-refractory iron deficiency anemia (9.3% of 518 patients with iron deficiency anemia). A complete screening for CD, AAG and the infection by Hp was made for 24 patients. A partial screening (only for one or two of these diseases) was made for 19 patients. In the 43 cases that at least one of the screenings were made, the prevalence of CD was about 4.7%, the prevalence of AAG was 14% and the infection by Hp was 30.2%. We couldn't evaluate the Hp eradication because it wasn't confirmed in six of 13 cases identified with this pathology. In the other seven cases, Hp eradication solved anemia in only one patient. Conclusion: We identified a high percentage of patients with iron-refractory iron deficiency anemia that suffer from AAG and infection by Hp, as is referred to in international studies. These findings reinforce the role of these diseases in iron-refractory iron deficiency anemia in the Portuguese population. This work reinforces the need to screen for CD, AAG and Hp infection in iron-refractory iron deficiency anemia.
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