Splenic B-cell lymphoma/leukemia with prominent nucleoli: A three-case series of the newly named old entity and review of literature.

CONTEXT: Splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN) aka hairy cell leukemia variant (HCL-v) is a rare B-cell chronic lymphoproliferative disorder. The main diagnostic challenge is to differentiate SBLPN from Classical hairy cell leukemia (HCL-c), as the former faces inferior responses to therapies and a poor prognosis. AIMS: The aim is to discuss the clinic-hematological and immunophenotyping findings of three cases of SBLPN. SETTINGS AND DESIGN: This is a retrospective observational study. METHODS AND MATERIAL: From the year 2011 to 2021, flow cytometry of all the cases with HCL diagnosis was reviewed, and three cases with negative or dim CD25 and hematological presentation matching with SBLPN were picked up. STATISTICAL ANALYSIS USED: Descriptive statistics is used. RESULTS: All the cases were male. The age ranges from 43 to 64 years. Median hemoglobin concentration, total leucocyte count, and platelet count were 8.6 g/dL, 6.9 × 109/L, and 53 × 109/L, respectively. The atypical cells were medium to large. All three showed prominent nucleoli. Bone marrow biopsies showed an interstitial pattern of infiltration in all the cases. The hairy cells were positive for CD20, CD11c, and CD103. CD25 was dim positive in one case. Annexin A1 was negative in all three cases. BRAF V600E mutation analysis was done in one case and turned out negative for the mutation. CONCLUSIONS: SBLPN is a rare entity, usually on-flow cytometry CD25 negative. However, in dim CD25-positive cases, BRAFV600E mutational analysis helps in discerning SBLPN diagnosis and differentiating it from HCL-c.

Plasma Cell Leukemia-Clinicopathological Profile from a Tertiary Care Center in Western India.

Poornima ManimaranIntroduction Plasma cell leukemia (PCL) is very uncommon and aggressive neoplasm constituting 2 to 4% of all plasma cell dyscrasias. By definition, clonal plasma cells should make up 20% of peripheral blood or have an absolute plasma cell count of 2 × 10 9 cells/cu.mm. PCL can be primary or secondary. In this study, the clinicohematological features of PCL, and correlation of immunophenotypic profile and conventional therapies with overall survival was analyzed. Materials and Methods This retrospective study involved PCL patients who were diagnosed across a 12-year period, from 2010 to 2021, at a tertiary care center in western India. Clinical, biochemical, peripheral smear, bone marrow aspirate, immunophenotyping, and molecular analysis were performed. Results Total 39 PCL patients were included in the study among which 36 were primary PCL patients. Splenomegaly (10/27), hepatomegaly (6/26), and lymphadenopathy (5/23) were noted. At presentation, all patients had anemia (<11g/dL), thrombocytopenia (33/39), hypercalcemia (>11mg/dl) 10/33 (30.3%) and lytic lesions was noted in 18/26 (69.2%). Immunophenotype of these patients showed CD 38 positivity, CD 138 positivity, CD56 positivity, and CD 117 negativity were 100, 62, 41.6, and 89%, respectively. Overall survival of our patients was 4.1 months and overall survival of patients treated with VTD (bortezomib, thalidomide, dexamethasone) and VCD (bortezomib, cyclophosphamide, dexamethasone) regimen was 3.4 and 4.1 months, respectively, which was not statically significant ( p -value 0.816). CD117 and CD56 markers were also not having any prognostic significance ( p -value 1.000 and 0.873, respectively). Conclusion Because of rarity of the disease, prospective studies are very limited and hence management and outcome of the disease are difficult to analyze. The current treatment protocols have no survival advantage and hence newer therapeutic approach is mandatory to attain better outcome.

A Comparative Analysis of Early T-Cell Precursor Lymphoblastic Leukemia/Lymphoma (ETP-ALL/LBL) and Non-ETP-ALL/LBL in a Tertiary Cancer Care Center Based in Western India.

Early T-cell precursor lymphoblastic leukemia (ETP-ALL) has a unique immunophenotype with very early T-cell differentiation. The current study summarises the distinct clinicopathological aspects of ETP-ALL and compares them with non-ETP-ALL. Twenty-nine ETP-ALL and 191 non-ETP-ALL cases were retrieved between 2018 and 2021. A P value was determined for each of the patient charaterisics (Table 1) to see for any significant relationship (P < 0.05) with ETP-ALL versus non-ETP-ALL. Kaplan-Meier log rank test was applied to look for any significant differences in OS for both the ALLs. ETP-ALL had an incidence of 12.6% out of total T-Acute lymphoblastic leukemia (T-ALL/LBL) in the past 3-years. Compared to non-ETP-ALL, ETP-ALL cases were associated with lower median age and male-to-female ratio. There was no statistically significant difference in the complete remission rate between both the subtypes. ETP-ALL was seen to be associated with high induction failure and relapse rate compared to non-ETP-ALL. To summarise, since the 2-year OS was poor compared to western research (for both ALLs), an intensive chemo-regimen should be implemented in the current situation. Some unusual markers were observed on flow-cytometry (ETP-ALL), which can be useful for MRD quantification, prognosis, and further trials for newer targeted therapies.

Untangling the Strands of Hairy Cell Leukemia: The Clinicopathological Spectrum over Eleven Years at a Tertiary Care Center.

BACKGROUND: Hairy Cell Leukemia (HCL) is an uncommon, indolent lymphoproliferative disorder of mature B lymphoid cells, accounting for 2% of all lymphoid tumors. The present study evaluated the clinical-hematological profile of HCL patients diagnosed at a single tertiary care center over a 11-year period. METHODS: The retrospective observational study was done between October 2010 and September 2021. The relevant clinical and laboratory information were retrieved from hospital medical records and electronic databases. The statistical analysis was performed using version 23.0 of SPSS. RESULTS: 66 (5.9%) of 1125 cases of chronic lymphoproliferative disorder were HCL. Splenomegaly was found in 47 (71.2%), hepatomegaly in 26 (39.5%), and lymphadenopathy in 17 (25.7%) of the cases. The mean hemoglobin, total leukocytes count, and platelets count were 8.04 g/dl, 6.76 X 109/L, and 77 X 109/L, respectively. Pancytopenia was detected in 40 cases (60.61 %). Bone marrow biopsies were majorly hypercellular and showed predominantly diffuse infiltration by atypical lymphoid cells. In two patients, initially thought of having refractory/hypoplastic anemia, the bone marrow biopsy and flow cytometry revealed HCL involvement.  42 cases of HCL underwent flow cytometry. CD20, CD 11c, CD 25 and CD 103 were positive in all the cases. The aberrant expression of CD5, CD10, and CD23 was found in frequencies of 5.71 %, 31.42 %, and 19.35%, respectively. In 40 cases for which follow-up information was available, there was full remission in 26 patients (65%), and later three showed relapse (7.5%) of which one died, and persistent leukemic activity in five (10%).  Eight patients (20%) died even before the initiation of treatment. One patient died within one month of therapy. No patient was examined for BRAF V600E mutation analysis. CONCLUSION: CD 10+ HCL was the most prevalent atypical immunophenotypic subgroup. Bone marrow biopsy and flow cytometry are crucial diagnostic tools to rule out hairy cell leukemia. However, BRAF V600E mutation analysis should be performed in cases with unusual presentation or resistance to treatment.

Acute Lymphoblastic Leukemia as Secondary Malignancy in a Case of Ewing's Sarcoma on Treatment.

The survival of Ewing's sarcoma (ES) has improved due to advances in both local and systemic therapy. This has given rise to an increased detection of second malignant neoplasms which can be in the form of solid tumors and hematological malignancies. The most common hematological malignancies are acute myeloid leukemia/myelodysplastic syndrome. Acute lymphoblastic leukemia (ALL) is relatively uncommon in occurrence in this setting. Furthermore, the average refractory period for hematological malignancies varies from 3 to 5 years. We report a case of a young female who developed ALL while on adjuvant therapy for ES.