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TO THE EDITOR: High-grade B-cell lymphoma (HG-BCL), an uncommon condition, displays a blastoid morphology and an aggressive disease course, but lacks the features of Burkitt’s lymphoma or diffuse large B-cell lymphoma [1, 2]. Previously termed “B cell lymphoma having features intermediate between DLBCL and Burkitt’s lymphoma” [3], its rarity has primarily resulted in descriptions of isolated case series.
We conducted a retrospective study on 14 patients diagnosed with HG-BCL who were registered in our department between January 1, 2020 and April 30, 2022. Their clinicopathological variables and outcomes at the end of the follow-up period are outlined in the Results.
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RESULTS
Epidemiology
The median age was 55.5 (range, 29–7) yr. Most patients were men (N=10, 71.4%). The common comorbidities were diabetes (N=3, 21.4%) and hypertension (N=2, 14.3%). The median symptom duration before diagnosis was eight (range, 1–20) wk. Seven (50%) patients did not have any B symptoms, whereas weight loss (N=3, 21.4%) and fatigue (N=4, 28.6%) were observed in the remaining seven (50%).
Disease workup
Bone marrow biopsy showed disease involvement in six (42.9%) patients. Central nervous system (CNS) involvement was confirmed in 1 out of 14 (7.1%) by a positive cerebrospinal fluid cytology. Four patients (28.6%) showed involvement of the spleen. Extra nodal site involvement was categorized as 1 site (N=7, 50%), ≥2 sites (N=4, 28.6%), and none (N=3, 21.4%). The median IPI and CNS IPI score was 3. High IPI (score of 4–5) and High CNS IPI (score of 4–6) were observed in three (21.4%) patients each.
The common stages observed were stages IV (N=8, 57.1%) and II (N=3, 21.4%). On IHC, five (35.6%) patients were found to be double-expressors of the markers C-MYC, BCL-2, and BCL-6. On analysis by FISH, C-MYC rearrangement was found in two (15.4%) patients with none having concurrent rearrangement of BCL-2 or BCL-6, as shown in Table 1.
Therapy
Nine (64.2%) patients received R-CHOP and five (35.7%) received R-DA-EPOCH as first-line therapy. CNS prophylaxis with intrathecal-methotrexate (IT-MTx) was administered concurrently with the R-DA-EPOCH regimen in all five patients, whereas five out of nine patients treated with R-CHOP received the same. High-dose MTx was administered to the remaining four patients on R-CHOP. Three (21.4%) patients relapsed and received salvage chemotherapy with R-GDP (N=2) and R-DHAP (N=1) regimens.
Treatment outcome
The median follow-up duration was 10.5 (1.13–19.3) mo. Response rates after first-line therapy were: complete response (CR), 71.4% (N=10); partial response (PR), 14.2% (N=2); stable disease (SD), none, and progressive disease (PD), 14.2% (N=2), with overall response rates (ORR) of 85.7%. One patient progressed rapidly on first line and died soon after starting therapy. Of the three (21.4%) patients who relapsed, two (14.2%) died from disease progression. The third death was due to COVID-19 pneumonia (Table 2).
Table 2
Clinical outcomes of patient cohort.
Survival
The mean progression-free survival (PFS) and overall survival (OS) were 14.86 (median, not reached) and 15.58 (median, not reached) mo, respectively. One-year PFS and OS were both 76.2%.
Adverse effects
Acute cytopenia (during and within 6 wk of treatment completion) observed in eight (57.1%) patients was the most common adverse effect. Maximum grade of toxicity was CTCAE 5 (N=3, 21.4%), whereas most common grade was CTCAE 2 (N=11, 78.5%).
To conclude, reports on HG-BCL data in India are scarce [4, 5]. Our study showed promising overall response and survival rates in patients with HG-BCL, despite most patients being in advanced disease stages. However, due to the small cohort size and short follow-up period, variables affecting the outcomes could not be studied. Multicenter pooling can provide greater insight into HG-BCL in the Indian context.
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