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  Table of Contents  
Year : 2019  |  Volume : 56  |  Issue : 4  |  Page : 373-374

Adult ALK-positive diffuse large B-cell lymphoma: A limited institutional experience

1 Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication11-Oct-2019

Correspondence Address:
Ajay Gogia
Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijc.IJC_705_18

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How to cite this article:
Vanidassane I, Gogia A, Mallick S. Adult ALK-positive diffuse large B-cell lymphoma: A limited institutional experience. Indian J Cancer 2019;56:373-4

How to cite this URL:
Vanidassane I, Gogia A, Mallick S. Adult ALK-positive diffuse large B-cell lymphoma: A limited institutional experience. Indian J Cancer [serial online] 2019 [cited 2020 Jun 1];56:373-4. Available from:

Anaplastic lymphoma kinase (ALK)–positive diffuse large B-cell lymphoma (DLBCL) is a rare subtype of DLBCL. There is scarcity of Indian data on this subtype, hence we retrospectively analyzed seven consecutive cases diagnosed with ALK DLBCL between January 2012 and January 2018, at Dr B. R. Ambedkar Institute, Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi.

Of seven patients, five were male, and the median age of the cases was 58 years (age range: 20–63 years). [Table 1] summarizes the clinical characteristics of the cases. Of the seven cases, six (86%) had an advanced stage disease (stage 3 or 4). International prognostic index score was 4 out of 5 for four patients. Four cases received cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP regimen), one case received rituximab along with CHOP (RCHOP), one case received cyclophosphamide, vincristine, and prednisolone (CVP), and one case received dose-adjusted etoposide, doxorubicin, cyclophosphamide, vincristine, and prednisolone (DA-EPCOH regimen). Only three patients achieved complete remission, one patients achieved partial remission, and three patients had primary progressive disease. Six patients had relapsed or progressed and succumbed to disease within 12 months from diagnosis. In refractory/relapsed cases, chemotherapy protocols like ICE (ifosfamide, carboplatin, etoposide) and CODOXM-IVAC (cyclophosphamide, vincristine, doxorubicin, methotrexate, alternating with ifosfamide, etoposide, and cytarabine) were used as salvage therapy. None of our patients underwent transplant as they were transplant-ineligible due to poor performance status or primary refractory disease. The median overall survival in our case series was 9 months (range: 3–26 months). Only one patient is alive without disease for 2.5 years at the time of analysis.
Table 1: Clinical characteristics and outcomes of ALK-positive DLBCL cases

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ALK-positive DLBCLs are consistently positive for CD138, MUM1, and EMA while usually negative for CD30. ALK-positive DLBCLs rarely express the usual B-lineage (e.g., CD19, CD20, CD79a) or T-lineage markers (e.g., CD2, CD3, CD7).

The disease response to standard CHOP is not encouraging unlike other DLBCL. The CD20-positive ALK-positive DLBCL is rare. In our series, one patient had CD20-positive ALK-positive DLBCL and she was treated with RCHOP and achieved complete remission. She is the only long-term survivor in our series. CD 20 positivity and addition of targeted agent like in the case could be attributed to good response. The role of rituximab in CD20–negative ALK-positive DLBCL is still unclear. As our previous experience with CHOP in this subtype was not satisfactory, one patient was treated with doseadjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (DA-EPOCH). The patient initially achieved a partial response but had disease progression and died of disease within a year of diagnosis. A study reported improved survival with EPOCH/CHOEP regimen; however, it was statistically insignificant.[1] Pan et al.[2] reported a 5-year overall survival of only 8% in advanced stage. Our experience similar to the available evidence, shows poor prognosis in this subset of the patient.[1],[2]

Recently, Wass et al.[3] reported a case of primary refractory ALK-positive DLBCL, who achieved a short remission with crizotinib. A study also showed that ALK expression in DLBCL is strictly linked to STAT3 phosphorylation and STAT3 inhibition is being explored as a treatment option in this aggressive group.[4]

To conclude, ALK-positive DLBCL is a rare aggressive subtype of DLBCL with poor prognosis, and it is evident that the current management of ALK-positive DLBCL is highly inadequate. Upfront high-dose chemotherapy with stem-cell rescue in chemosensitive disease targeted agents like rituximabin CD20-positive cases, and brentuximabin CD30-positive cases should be explored in this subgroup. Better understanding of the biology of this lymphoma is required to explore various therapeutic targets in future.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Jiang XN, Yu BH, Wang WG, Zhou XY, Li XQ. Anaplastic lymphoma kinase-positive large B-cell lymphoma: Clinico-pathological study of 17 cases with review of literature. PLoS One 2017;12:e0178416.  Back to cited text no. 1
Pan Z, Hu S, Li M, Zhou Y, Kim YS, Reddy V, et al. ALK-positive large B-cell lymphoma: A clinicopathologic study of 26 cases with review of additional 108 cases in the literature. Am J Surg Pathol 2017;41:25-38.  Back to cited text no. 2
Wass M, Behlendorf T, Schädlich B, Mottok A, Rosenwald A, Schmoll HJ, et al. Crizotinib in refractory ALK-positive diffuse large B-cell lymphoma: A case report with a short-term response. Eur J Haematol 2014;92:268-70.  Back to cited text no. 3
D'Amore ES, Visco C, Menin A, Famengo B, Bonvini P, Lazzari E.STAT3 pathway is activated in ALK-positive large B-cell lymphoma carrying SQSTM1-ALK rearrangement and provides a possible therapeutic target. Am J Surg Pathol 2013;37:774-86.  Back to cited text no. 4


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