Objectives
Pediatric myelofibrosis is a rare entity with the largest reported series of 19 cases. We describe here the clinicopathological spectrum and outcomes of 15 cases of pediatric myelofibrosis.
Methods
Case files of myelofibrosis of patients less than 18 years were retrieved from January 2016 to January 2019, and patients with idiopathic myelofibrosis after exhaustive work‐up were studied. Their clinicopathological profiles were studied and then followed up for resolution and malignant transformation.
Results
Of the 15 cases of idiopathic myelofibrosis, transfusion‐dependent anemia (14/15) was most common presentation. Only one patient showed leukoerythroblastosis with dacryocytes. Myeloid hyperplasia was seen in 13 of 15 patients and megakaryocytic hyperplasia in 10 patients. Dysmegakaryopoiesis was seen in 8 of 15 patients, and only three had small loose megakaryocytic clustering. None showed hyperchromatic megakaryocytes, intrasinusoidal hematopoiesis, or osteosclerosis. One patient with trisomy 8 tested positive for JAK2V617F. Bone marrow biopsy was hypercellular in 13, and 8 had world health organization (WHO) MF‐3 fibrosis. None of the patients developed malignancy, one had spontaneous resolution, and one patient required allogenic stem cell transplant.
Conclusions
Pediatric myelofibrosis is a distinct entity from primary myelofibrosis in adults and merits mention in the WHO manual as a distinct entity.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal stem cell disorder. Eculizumab and bone marrow transplantation are disease‐modifying treatments for PNH but may not be readily available in resource‐constrained settings. Of 52 pediatric patients with PNH, 20 had classical PNH and 32 had PNH/aplastic anemia (PNH/AA). Median time to diagnosis was 30 months in classical PNH patients. Renal failure was present in four patients (20%). Six (30%) achieved complete response, 10 (50%) achieved partial response with androgens in classical PNH. Two underwent allogenic stem cell transplantation. In the PNH/AA group, 16 (50%) were in CR and seven (21%) were in PR with anti‐thymocyte globulin ± cyclosporine.
Letter to Editor: Pediatric Hodgkin lymphoma (HL) rarely presents with central nervous system (CNS) involvement at the time of initial presentation. Rather, it is more often seen in patients who have relapsed. We report a child with HL who had CNS involvement at initial presentation.A 6-year-old female presented with complaints of fever for 5 months, jaundice, and multiple nodular swellings in the neck and axilla for 3 months. On physical examination, she was febrile, pale, and icteric with cervical and axillary lymphadenopathy with largest lymph node 2 × 2 cm and massive hepatosplenomegaly.Laboratory investigations revealed anemia (hemoglobin 8.8 g/dl), total leucocyte count 5.2 × 10 9 /l, platelet count 256 × 10 9 /l, and abnormal liver function tests. Fine-needle aspiration cytology from the axillary node revealed scattered histiocytic clusters and atypical cells, with high nuclear-cytoplasmic ratio, hyperchromatic nuclei, and small nucleoli and the same was confirmed on biopsy. Immunohistochemistry displayed CD15 and CD30 positivity, which was consistent with the diagnosis of Hodgkin lymphoma, mixed cellularity type. Bone marrow biopsy showed infiltration with the tumor cells. Contrastenhanced computed tomography scan of the chest and abdomen revealed mediastinal and mesenteric lymphadenopathy along with hepatosplenomegaly.
Previous studies have shown the vulnerability of hematological patients with the Coronavirus disease of 2019 (COVID-19). We aimed to compare the outcomes and risk factors for poor survival in patients with hematological conditions hospitalized with COVID-19 infection. Single centre, retrospective, cohort study included all patients with a hematological condition admitted to Rajiv Gandhi Cancer Institute & Research Centre, New Delhi, India between 1 April 2020 and 31 May 2021. Of a total of 154 patients, 81 were in the pre-delta group and 73 were in the delta group out of which 21 (25.97%) in the pre-delta group and 24 (33.88%) patients in the delta group died. Haematological characteristics—age > 60 years, progressive hematological cancer, more than two lines of anti-cancer therapy, and active chemo-immunotherapy or targeted therapy were associated with higher mortality in the delta group. COVID-19 characteristics associated with higher mortality during the delta wave were severity of COVID infection, higher oxygen requirements, and COVID plasma therapy There were no deaths in individuals (n = 15) within the delta group who received COVID-19 vaccination. This study adds to the evidence that patients with hematological diseases are a particularly vulnerable group and the delta variant of the virus is associated with higher mortality. We could identify patient characteristics and features related to COVID-19 infection and underlying hematological conditions that were associated with poor outcomes in the delta sub-group. Vaccination was found to be an effective strategy for overcoming mortality and morbidity in these patients.
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