Plasmablastic Lymphoma in a Human Immunodeficiency Virus-positive Child With a Suspicion of Concomitant Primary Immunodeficiency Disorder.

Plasmablastic lymphoma (PBL) occurs in the setting of immunodeficiency, in association with human immunodeficiency virus (HIV) infection, in elderly patients, and in the posttransplantation state. It is exceptionally rare in children. PBL is an aggressive lymphoma with a poor prognosis. We present a case of pediatric PBL in an HIV-positive child with suspicion of a concomitant underlying immune deficiency state other than HIV. A 7-year-old girl presented to the pediatric emergency department with complaints of fever and painful swelling on the left side of her face for 15 days, associated with headache, snoring, and difficulty in breathing. She had a history of watery diarrhea, oral thrush, recurrent fever, and hospitalizations for skin infections since the age of 1 year. Histopathological findings were consistent with PBL. Her HIV RNA polymerase chain reaction was positive. She was offered chemotherapy based on the FAB/LMB 96 protocol. This case demonstrates an aggressive presentation of a rare entity, HIV-associated PBL, in a child, with underlying immunodeficiency and highlights the issues which caused a significant challenge in making the diagnosis. The presence of HIV infection and contradicting other immunologic investigations posed a dilemma in establishing an association of PBL in this child. The outcome of patients with this tumor is associated with high mortality.

Tibial Plasmablastic Lymphoma in a HIV-Negative Child: A Novel Extraoral Localisation.

Plasmablastic lymphoma is an aggressive, high-grade non-Hodgkin lymphoma predominantly seen in HIV-infected individuals. Alongside a strong correlation with HIV, PBL can manifest in immunocompromised HIV-negative patients. A rare case of PBL in an immunocompetent and otherwise healthy child presented to Indus Hospital & Health Network (IHHN), Karachi, Pakistan. The patient had complaints of swelling and pain in the right leg and was referred from a city in Interior Sindh. Histopathological analysis revealed sheets and aggregates of neoplasm replacing bone marrow interspersed with sclerotic bony fragments. Large, monomorphic, multinucleated neoplastic cells containing abundant cytoplasm and scattered pleomorphic cells were also noted, leading to the diagnosis of tibial plasmablastic lymphoma. A FAB/LMB96 group C chemotherapy regimen for aggressive and high-risk cancer was administered with a marked improvement in clinical symptoms.

An Outcome Analysis of Childhood Acute Promyelocytic Leukemia Treated with Atra and Arsenic Trioxide, and Limited Dose Anthracycline.

The overall survival of Acute Promyelocytic Leukemia (APL), reported in recent studies, is approaching to 90% wherein, arsenic trioxide (ATO) and all-trans retinoic acid (ATRA) are used as the mainstay of treatment with either limited or no use of anthracycline and cytarabine. This study is aimed to ascertain the outcome of children with APL using similar approach. A total of 30 patients with APL, registered from January 2015 to December 2018, were reviewed. Diagnosis was established on bone marrow aspirate and confirmed by the presence of PML-RARA translocation. Treatment protocol was based on Australian APML 4 study performed by Australian Leukemia Lymphoma Group (ALLG). Lumbar puncture was not performed as it was not part of the protocol due to the risk of bleeding. The mean age in current cohort was 9 years with 53% males. Seven (23.3%) patients died and three (10%) abandoned treatment during induction. Twenty patients completed the intensive phase of chemotherapy and all (100%) of them attained molecular remission (MR). One patient dropped out after MR whereas, 19 remain on follow up with no evidence of disease, reflecting disease free survival (DFS) of 95%. With a median follow up of 2.5 years (range 2.1-4.8 years) the 5 years Kaplan-Meier estimate of OS was 63% and 73%, with and without abandonment, respectively. Analysis of outcome according to risk groups revealed inferior outcome of high risk (HR) group (38% and 50% with and without abandonment, respectively) in contrast to standard risk (SR) group which showed better outcome (82% and 88% with and without abandonment, respectively). The attainment of 100% molecular remission and absence of relapse supports the effectiveness of this regimen. Moreover, it is found to be less toxic and therefore, can be conveniently managed in day-care settings.

Hypercalcemia and Disseminated Osteolytic Lesions With Normal Blood Counts and Absence of Circulating Blasts: A Rare Presentation of Childhood B-Lymphoblastic Leukemia.

Hypercalcemia and disseminated osteolytic bone lesions are a rare presentation of pediatric acute lymphoblastic leukemia (ALL). The authors report a 3-year-old boy who presented with hypercalcemia and diffuse osteolytic lesions involving axial and appendicular bones. He had normal complete blood count and the absence of blasts in peripheral smear; however, bone marrow aspirate and trephine were consistent with B-cell ALL. A review of the literature highlights the variable clinical outcome of this rare presentation depending on the presence of hypercalcemia and osteolytic lesions with or without chromosomal translocation t(17;19) and coagulation abnormalities. The patient had no coagulopathy and normal karyotype, and showed excellent response to initial treatment in terms of complete remission and negative minimal residual disease after standard-risk induction chemotherapy. Hypercalcemia with diffuse osteolytic lesions warrants bone marrow examination to rule out leukemia even in the absence of any abnormality in complete blood count. The case was reported for awareness of this rare presentation of ALL so that delays can be avoided for this potentially curable but life-threatening disease.