The Outcome of Pediatric Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia: Experience from a Referral Center in South India.

Although improved survival in children with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph-ALL) has been demonstrated in trials, the outcome appears to be inferior in low- and middle-income countries (LMIC). Methods A file review of children aged ≤ 15 years diagnosed with Ph-ALL from 2010 to 2019 was performed. Minimal residual disease (MRD) was assessed by flow-cytometry. Real-time polymerase chain reaction (qRT-PCR) was used to quantify the BCR::ABL1 transcripts during treatment. Results The mean age of the 20 patients in the study was 91 months. Of 19 patients in whom the BCR::ABL1 transcript was confirmed, 10(50%) had P210, 7(35%) had P190, and two showed dual expression. The mean dose of imatinib that was administered was 294 ± 41 mg/m2/day. qRT-PCR for BCR::ABL1 was < 0.01% in all patients who were in remission or had a late relapse and was ≥ 0.01% in patients who had an early relapse. Two patients underwent HSCT. The 3-year event-free survival (EFS) was 35.0 ± 10.7%. Patients with a good prednisolone response (GPR) and a negative end-of-induction MRD demonstrated a superior EFS to those who lacked either or both (80.0 ± 17.9% vs. 16.7 ± 15.2%, P = 0.034). Conclusion The 3-year EFS of 20 children with Ph-ALL treated with chemotherapy and TKI was < 50%. An unusually high proportion of patients with p210 transcript expression; sub-optimal TKI dosing and lesser intensity of chemotherapy, due to the concern of high treatment-related mortality in LMIC are possible reasons for the poor outcome. Conventional treatment response parameters such as GPR and MRD predict outcomes in Ph-ALL. qRT-PCR for BCR::ABL1 may have a role in predicting early relapse. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-023-01684-9.

What happens to children with acute lymphoblastic leukemia in low- and middle-income countries after relapse? A single-center experience from India.

Managing a child with acute lymphoblastic leukemia (ALL) after relapse is arduous in low- and middle-income countries. A file review of children aged ≤15 years diagnosed with relapsed ALL from 2010 to 2019 was performed. Classification of relapse followed the Berlin-Frankfurt-Münster (BFM) scheme. The majority of patients were treated with a modified ALL-REZ-BFM protocol. Of 764 children treated for ALL in the study period, 163 (21.3%) relapsed. The median age at relapse was 101 months (range: 8-297). The immunophenotype was B-ALL and T-ALL in 140 (86%) and 23 (14%) patients. The site of relapse was extramedullary, combined, and medullary in 46 (28%), 45 (28%), and 72 (44%) patients. Very early, early, and late relapses were observed in 57 (35%), 66 (40%), and 40 (25%) patients. The proportions of extramedullary and medullary sites were greater among patients with early and late relapses, respectively (p = 0.039). Eighty-four (52%) patients were treated with palliative intent. The 2-year event-free survival (EFS) of patients treated with curative intent was 36.3 ± 6.3%. The 2-year EFS for very early/early and late relapses were 18.2 ± 6.2% and 67.6 ± 10.4% (p < 0.001). The 2-year EFS did not differ between extramedullary, combined, and medullary relapses. Treatment-related mortality occurred in 14 (20%) patients. More than 50% of the patients with relapse were treated with the intent of palliation. Extramedullary relapses were more likely to be early and did not have a better outcome than medullary relapses. Children with late relapse had a fair chance of survival with chemotherapy.

Role of Hysterosalpingography in Diagnosing Tubal Blockage - A Prospective Diagnostic Study.

BACKGROUND: Women with abnormal hysterosalpingography (HSG) are anxious regarding the presence of tubal pathology. It is important to know the predictive value of HSG and the need for subsequent laparoscopy following an abnormal report. In the era of assisted reproductive technology, the role of invasive testing such as diagnostic laparoscopy is being increasingly questioned due to its invasiveness and associated risks. There is a need to explore the positive predictive value (PPV) of HSG in detecting bilateral tubal block in our population as PPV changes with the prevalence of disease. AIM: The aim of this study was to evaluate the diagnostic accuracy of HSG in identifying tubal blockage in subfertile women. SETTING AND DESIGN: This was a prospective diagnostic study conducted in the department of reproductive medicine and surgery in a university-level hospital. MATERIALS AND METHODS: The study included 199 subfertile women who had undergone HSG earlier and were planned for laparoscopy from April 2017 to January 2021. Findings of HSG and laparoscopy were compared with HSG as index test and laparoscopy as reference test, and the outcomes analysed were PPV of HSG for a bilateral tubal block, bilateral hydrosalpinx, abnormal HSG (unilateral or bilateral tubal block) and agreement between HSG and diagnostic laparoscopy in detecting normal and abnormal findings. STATISTICAL ANALYSIS: Kappa statistics, Stuart-Maxwell tests of marginal homogeneity and prevalence-adjusted bias-adjusted kappa (PABAK) statistics were used. RESULTS: The PPV for a bilateral block with HSG was 20.9% (95% CI: 13.7-29.7). The PPV of HSG for bilateral hydrosalpinx was 50.0% (95% CI: 6.8-93.2). PABAK was estimated to be 0.42 (95% CI: 0.30-0.55), suggestive of moderate agreement between the tests. Findings of laparoscopy in women with at least one patent tube in HSG showed that in 12.3% of cases, the management was likely to change due to the operative findings. CONCLUSION: The current study showed low PPV for bilateral tubal block diagnosed with HSG which translates into a need for further confirmation by laparoscopy. In one out of every eight women with at least one patent tube on HSG, performing laparoscopy changed the management.