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Biomedical subjects

Sameer Bakhshi

Publications and source records attributed to Sameer Bakhshi.

At least 19 recordsLinked to original sources

Pleomorphic rhabdomyosarcoma, outcomes of patients with advanced disease treated with systemic agents: Retrospective study from the global pushing ultra-rare sarcomas towards hope (PUSH) consortium.

OBJECTIVES: To report the outcomes in adult patients with advanced pleomorphic rhabdomyosarcoma (P-RMS) treated with systemic therapy. METHODS: This global, multicenter, retrospective study conducted within the Pushing Ultra-Rare Sarcomas Towards Hope consortium (PUSH) included patients > 40 years with histologically confirmed advanced P-RMS, treated with at least one line of systemic therapy between 2013 and 2023. The primary endpoint was progression-free survival from first diagnosis of advanced disease, and from systemic treatment start (PFS-1 and PFS-2). Secondary endpoints included overall response rate (ORR), overall survival from first diagnosis of advanced disease and from treatment start (OS-1 and OS-2), and treatment-specific outcomes. RESULTS: Seventy-seven patients were included from 21 sarcoma reference centers. At a median follow-up of 44 months (IQR: 17.0-74.8), 49 (64%) patients had died and 48 (62%) had progressed. The median OS-1 and PFS-1 were 13.6 (95% confidence interval (CI): 9.4-22.5) and 5.4 (95% CI: 4.2-7.3) months, respectively. Two- and three-year OS-1 were 32.5% and 30.3%. Anthracycline-based regimens (n = 42) achieved a 50% ORR, with mPFS-2 and mOS-2 of 5.2 and 19.2 months; gemcitabine-based regimens (n = 15) a 42% ORR, with mPFS-2 and mOS-2 of 3.7 and 7.8 months; pazopanib (n = 6) a 33% ORR, with mPFS-2 and mOS-2 of 2.4 and 4.2 months; PD-1 inhibitors (n = 2) induced one response lasting 53 months. CONCLUSIONS: This series of advanced P-RMS treated with systemic agents, the largest available to date, showed meaningful activity of anthracycline- and gemcitabine-based regimens, and anecdotal responses to pazopanib and PD-1 inhibitors. Further prospective validation is planned.

Humans↗

A case report of a patient with microcephaly, facial dysmorphism, mitomycin-c-sensitive lymphocytes, and susceptibility to lymphoma.

We report on a 17-year-old boy with a unique lymphocyte mitomycin-C (MMC)-sensitive chromosomal breakage syndrome. He had failure to thrive, and has microcephaly, slight facial dysmorphism, and constitutional short stature but no other phenotypic or hematological manifestations of Fanconi anemia (FA). He developed B-cell lymphoma of the neck, which was treated with standard doses of alkylating agents. Major side effects related to chemotherapy did not occur. Normal erythrocyte corpuscular volume, MMC-insensitive fibroblasts, and the occurrence of lymphoma rather than AML sets this patient apart from typical FA. The combination of constitutional dwarfism, microcephaly, MMC-sensitive lymphocytes, and susceptibility to lymphoma represents an unusual constellation of symptoms among genetic disorders.

Adolescent↗

Hodgkin's disease in Indian children: outcome with chemotherapy alone.

BACKGROUND: To assess the efficacy of chemotherapy alone, using four cycles of COPP alternating with four cycles of ABVD in all stages of childhood Hodgkin's disease (HD). PROCEDURE: Between January 1991 and February 2001, 148 previously untreated patients were investigated, treated, and analyzed for remission and survival. RESULTS: There were 134 boys and 14 girls with a median age of 8 years, 75% were less than 10 years old. 63.5% had advanced stage disease (IIB-IV). B symptoms were present in 54.4% of cases; bulky mediastinal mass in 18 cases (12.2%); spleen and bone marrow involvement in 22 (14.9%) and four cases (2.7%), respectively. Mixed cellularity (MC) subtype was found in 86.0%. Response to treatment was evaluated in 133 patients: complete remission (CR) was achieved in 121 patients (91.0%), partial remission (PR) in seven (5.3%), progression occurred in two (1.5%), and three (2.3%) died on therapy. Four patients with mediastinal residual disease were given additional involved field radiotherapy. Out of 111 patients analyzable, five (4.5%) have relapsed 6-30 months after completing chemotherapy, and were treated with additional cycles of ABVD and low-dose involved field radiotherapy. The 5-year actuarial overall survival (OS) and event-free survival (EFS) are 91.5 and 87.9%, respectively. Advanced stage, B symptoms, anemia, spleen, and marrow involvement were adverse prognostic factors for survival. CONCLUSIONS: Chemotherapy alone with alternating COPP/ABVD, without additional radiotherapy, provides high rates of durable remission and is an effective therapy in childhood HD, even in case of large mediastinal mass and peripheral or abdominal bulky disease.

Adolescent↗

Acute myeloid leukemia with severe aplastic anemia following immunosuppressive therapy.

Severe aplastic anemia (AA) is a life-threatening condition wherein bone marrow transplantation (BMT) is the therapy of choice in a young patient who has a matched sibling donor. Here, we report an 11-year-old boy with severe AA who was referred for BMT late in its course when he had developed acute myeloid leukemia following two courses of immunosuppressive therapy with antithymocyte globulin and cyclosporin. He was then treated with induction therapy using cytosine arabinoside and daunomycin for acute myeloid leukemia, but he succumbed due to infection and refractory leukemia. We discuss the relevance of early referral for BMT in severe AA.

Anemia, Aplastic↗

Childhood primary mesenteric seminoma.

We report an 11-year-old child who presented with an abdominal lump and was diagnosed as having an extragonal primary mesenteric seminoma. Patient was treated with 4 cycles of combination chemotherapy cisplatin, etoposide and bleomycin; he is now disease free for 2 years. We discuss and review extragonadal germ cell tumors arising from the mesentery and their management.

Antineoplastic Combined Chemotherapy Protocols↗

Extensive pneumothorax, pneumomediastinum and surgical emphysema as a complication of bleomycin therapy.

Bleomycin is a commonly used chemotherapeutic agent and one of the commonest cytotoxic drugs leading to pulmonary parenchymal damage. It generally leads to interstitial pneumonitis and fibrosis, hypersensitivity reactions and acute respiratory distress syndrome. We describe an 8-year-old boy who, following prolonged bleomycin therapy, demonstrated extensive air dissection and extrapulmonary air, an unusual and fatal complication.

Antibiotics, Antineoplastic↗

Invasive colonic mucormycosis in early induction therapy of childhood acute lymphoblastic leukemia.

Fungal infections occur in patients who are severely immunocompromised with profound and prolonged neutropenia. We report a patient of acute lymphoblastic leukemia who developed nonspecific abdominal symptoms within two days after the onset of neutropenia in the early induction phase of chemotherapy, which was later found to be due to intestinal mucormycosis and resulted in a fatal outcome.

Antineoplastic Combined Chemotherapy Protocols↗

Transthoracic lung aspiration in etiology of pneumonia.

Transthoracic lung aspiration was performed in 30 episodes of pneumonia in 27 children with malignancy on chemotherapy to assess etiology of pulmonary infections. Total of 22 organisms were isolated in 16/30 (53.3%) episodes. No acid fast bacilli or Pneumocystis carinii were seen. Organisms grown by blood culture correlated with that of lung puncture in 5 episodes, while throat culture and nasopharyngeal organisms correlated with that of lung puncture on one occasion each. Organisms isolated in 8/18 episodes (44.4%) of antemorten transthoracic aspiration included: Pseudomonas aeruginosa, Klebsiella pneumoniae, Escherichia coli, Streptococcus faecalis and Diphtheroids. In 3/18 episodes, lung puncture results altered treatment and thus resulted in survival of the patients. Only one minor complication occurred in this study--pneumothorax that resolved spontaneously. Thus, transthoracic lung puncture is an useful and safe procedure in immunocompromised patients with pneumonia who do not respond to initial broad spectrum antibiotics.

Adolescent↗

Autoimmune hemolytic anemia complicating disseminated childhood tuberculosis.

An 8-year-old girl presented with severe autoimmune hemolytic anemia (AIHA) in association with mediastinal widening. Biopsy of mediastinal lymph node confirmed the diagnosis of tuberculosis. A diagnosis of disseminated tuberculosis in association with AIHA was made, and the patient was treated with steroids and antitubercular therapy. This is the first report case of AIHA in association with childhood tuberculosis; we also discuss other reported cases of AIHA in association with adult tuberculosis in English literature.

Anemia, Hemolytic, Autoimmune↗

Bone relapse in acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) can occasionally relapse in unusual extramedullary sites like bone. Here we present a 6.5-year old boy with 'T' cell ALL who developed a swelling in left tibia which was infiltrated with lymphoblasts 7 months after completion of chemotherapy. Bone marrow and cerebrospinal fluid were negative for blasts. This is the first reported case of bone relapse in ALL from India. We discuss the previous cases of isolated bone relapse in ALL reported in English literature.

Bone and Bones↗

Breast feeding and childhood hematological malignancy.

Breast milk is known to have anti-infective and immunomodulating effects on infants, but its association with childhood cancer has not been well studied. Artificial feeding may affect the immune response in carcinogenesis. In this communication the authors have reviewed different articles describing the association between breast feeding (BF) and subsequent development of childhood hematological malignancy. It appears that BF may have a protective effect on childhood cancer, both the duration of BF as well as the quantity of milk ingested is probably critical to the beneficial immunological effects of BF against childhood cancer if any.

Adolescent↗

Non hodgkin's lymphoma seven years following remission of acute lymphoblastic leukemia.

The authors describe a case of extramedullary relapse in lymph node presenting as lymphoblastic lymphoma seven years following remission of acute lymphoblastic leukemia. To the best of our knowledge, this is the first reported case of an isolated lymph node relapse with hematopoietic remission of leukemia. We have discussed cases of large cell lymphoma and other unusual areas of extramedullary relapse complicating acute lymphoblastic leukemia in hematopoietic remission.

Antineoplastic Combined Chemotherapy Protocols↗

Rathke's cleft cyst: missed etiology of panhypopituitarism in presence of coexistent ocular and neurocysticercosis.

We report a 10 year-old girl with panhypopituitarism and coexistent ocular and neurocysticercosis. Intrasellar cystic lesions whether neoplastic or non-neoplastic in origin are often difficult to distinguish because their symptoms, signs, and radiological characteristics are similar. The diagnosis of intrasellar cysticercosis was initially considered because of high endemicity, positive serology for cysticercus and radiological evidence of cysticercosis in the eye and parietal lobe. However, since there was no improvement with cysticidal therapy and no radiological resolution of the sellar lesion, surgery was performed, which revealed a Rathke's cleft cyst.

Central Nervous System Cysts↗

Near-triploid acute lymphoblastic leukemia with TEL/AML1 translocation.

An 11-year-old girl was diagnosed as B-precursor acute lymphoblastic leukemia (ALL) with co-expression of myeloid antigens. Cytogenetic analysis revealed a near-triploid (75-82 chromosomes/cell) abnormal chromosomal complement. Fluroscent in situ hybridization studies indicated presence of TEL/AMLI fusion genes. We discuss the prognostic relevance of TEL/AML1 in this rare near-triploid subtype of ALL.

Burkitt Lymphoma↗

Significance of splenomegaly in childhood Hodgkin disease.

OBJECTIVES: Spleen involvement in childhood Hodgkin disease (HD) has not been described extensively. The authors' purpose was to study the clinicoradiologic features and prognostic significance of splenomegaly and that of splenic involvement in childhood HD. METHODS: One hundred forty-one children presenting with HD between January 1991 and February 2001 and treated with chemotherapy alone (4 COPP/4 ABVD) were included in the study. Radiotherapy was given in four patients with residual mediastinal disease. Patients were staged clinically and assessed for splenic deposits by computed tomography, ultrasonography, or both. RESULTS: Splenic involvement was seen in 22 children (15.6%). On univariate analysis, spleen deposits were significantly correlated with constitutional symptoms (P = 0.02), splenomegaly on physical examination (P < 0.001), involvement of three or more lymph node areas (P = 0.006), involvement of subdiaphragmatic lymph nodes (P = 0.01), mediastinal involvement (P = 0.001), and bulky disease (P = 0.005). Multivariate analysis retained enlarged spleen, involvement of three or more lymph node areas, and bulky disease as significant risk factors for spleen involvement. Relapse occurred significantly more in children with splenic involvement (P = 0.04) and in those with splenomegaly (P = 0.04). Presence of splenic deposits was an adverse prognostic factor for 5-year event-free survival (67.5% vs. 93.0%, P = 0.01). CONCLUSIONS: Enlarged spleen, involvement of three or more lymph node areas, and bulky disease are predictable risk factors for HD splenic deposits. Splenic involvement on computed tomography scan or ultrasonography is significantly associated with relapse and contributes to a poorer outcome of HD in children treated with chemotherapy alone.

Adolescent↗