CC BY-NC-ND 4.0 · South Asian J Cancer
DOI: 10.1055/s-0041-1728224
Original Article

Adult Philadelphia-Positive Acute Lymphoblastic Leukemia: A Single-Institution Experience in Limited-Resource Setting

Rudresha Haleshappa Antapura
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Amale Baburao Vaibhav
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Lokanatha Dasappa
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Linu Abraham Jacob
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Mallekavu Channappa Sureshbabu
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Kadabur Nagendrappa Lokesh
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Lakkavalli Krishnappa Rajeev
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Smitha C. Saldanha
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
,
Tirumala Venkatesh
1   Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
› Author Affiliations
Funding None.

Abstract

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L. K. Rajeev

Background Adult Philadelphia-positive (Ph + ) acute lymphoblastic leukemia (ALL) is a distinct entity with poor prognosis. Treatment with tyrosine kinase inhibitors improved responses but still with poor outcomes. We evaluated treatment outcomes in these patients treated in limited-resource settings in the absence of availability of allogeneic stem cell transplantation (ASCT).

Materials and Methods We studied case record files of the adult patients diagnosed with Ph+ ALL.

Results A total of 18 patients were evaluated retrospectively. The median age of presentation was 28 years. Male-to-female ratio was 1:1. Patients presented with fever and fatigue. Six patients (33.33%) presented with cervical lymphadenopathy. Clinical splenomegaly was present in 16 (88.88%) patients on palpation, whereas on ultrasonographic evaluation, all 18 patients had splenomegaly. The median size of the spleen was 15 cm. Hepatomegaly was seen in 5 (27%) patients. All 18 patients had anemia at the time of presentation. Leukocytosis was seen in 17 (94.44%) patients, whereas 1 (5.56%) patient presented with low total leukocyte count. The median platelet count at the time of presentation was 30,000/mm.3 On peripheral smear, median number of blast cells was 55%, and on bone marrow aspiration samples, median blast percentage seen was 70%. Conventional cytogenetics was done in all the patients on bone marrow aspiration samples. Ten patients (55.55%) had t(9;22) – Ph chromosome. One patient (5.56%) on cytogenetics showed double Ph chromosome. The median value of breakpoint cluster region-ABL1 transcript in IS% was 13%. Seventeen (94.44%) received ALL protocol (BFM95) along with tyrosine kinase inhibitor (imatinib). One (5.56%) patient refused aggressive cytotoxic chemotherapy. No patient underwent ASCT. The median duration of follow-up was 7.5 months, ranging from 3 to 16 months. Median overall survival (OS) was 7.5 months and 2-year OS was 33.33%.

Conclusion Poor prognosis of this disease, especially in the absence of ASCT, remains a major challenge in the treatment.



Publication History

Article published online:
10 April 2023

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  • References

  • 1 Arber DA, Orazi A, Hasserjian R. et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127 (20) 2391-2405
  • 2 Pullarkat V, Slovak ML, Kopecky KJ, Forman SJ, Appelbaum FR. Impact of cytogenetics on the outcome of adult acute lymphoblastic leukemia: results of Southwest Oncology Group 9400 study. Blood 2008; 111 (05) 2563-2572
  • 3 Chiaretti S, Vitale A, Cazzaniga G. et al. Clinico-biological features of 5202 patients with acute lymphoblastic leukemia enrolled in the Italian AIEOP and GIMEMA protocols and stratified in age cohorts. Haematologica 2013; 98 (11) 1702-1710
  • 4 Terwilliger T, Abdul-Hay M. Acute lymphoblastic leukemia: a comprehensive review and 2017 update. Blood Cancer J 2017; 7 (06) e577
  • 5 Liu-Dumlao T, Kantarjian H, Thomas DA, O'Brien S, Ravandi F. Philadelphia-positive acute lymphoblastic leukemia: current treatment options. Curr Oncol Rep 2012; 14 (05) 387-394
  • 6 Fielding AK, Rowe JM, Buck G. et al. UKALLXII/ECOG2993: addition of imatinib to a standard treatment regimen enhances long-term outcomes in Philadelphia positive acute lymphoblastic leukemia. Blood 2014; 123 (06) 843-850
  • 7 Ravandi F. How I treat Philadelphia chromosome-positive acute lymphoblastic leukemia. Blood 2019; 133 (02) 130-136
  • 8 Danthala M, Gundeti S, Maddali LS, Pillai A, Puligundla KC, Adusumilli RP. Philadelphia chromosome-positive acute lymphoblastic leukemia: 8 years' experience from a tertiary care center in India. South Asian J Cancer 2016; 5 (04) 176-178
  • 9 Jain P, Korula A, Deshpande P. et al. Adult acute lymphoblastic leukemia: limitations of intensification of therapy in a developing country. J Glob Oncol 2018; 4: 1-12
  • 10 Lim SN, Joo YD, Lee KH. et al. Long-term follow-up of imatinib plus combination chemotherapy in patients with newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia. Am J Hematol 2015; 90 (11) 1013-1020