Clinical Features and Outcomes of the Patients with B-Cell Chronic Lymphoproliferative Disease in the New Drug Era / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical characteristics of the patients with B-cell chronic lymphoproliferative disease（B-CLPD） in the new drug era and the effect of new drug treatment on efficacy and survival.@*METHODS@#The clinical and laboratory data of 200 cases B-CLPD patients diagnosed between April 2015 and August 2021 were analyzed retrospectively. The clinical efficacy and survival of the patients under different treatments including Bruton tyrosine kinase（BTK） inhibitors， rituximab， and chemotherapy alone were analyzed. The prognostic factors affecting the survival of patients were analyzed by univarite analysis and multivariate analysis.@*RESULTS@#There were 119 male（59.5％） and 81 female（40.5%） in 200 cases B-CLPD patients， the sex ratio(male/female) was 1.5∶1 with median age of 61（30- 91） years old. The distribution of subtypes were as fallows: 51 cases (25.5%) of chronic lymphocytic leukemia/small lymphocytic lymphoma（CLL/SLL）， 64（32.0%） cases of follicular lymphoma（FL）， 40（20.0%） cases mantle cell lymphoma（MCL）， 30（15.0%） cases of marginal zone lymphoma（MZL）， 10（5%） cases of lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia（LPL/WM）， 5（2.5%） cases of B cell chronic lymphoproliferative disorders unclassified（B-CLPD-U） . The main clinical manifestation of 102 patients was lymph node enlargement, 32 cases were complicated with B symptoms. Among CLL/SLL patients， there were 12（23.5%） cases in Binet A and 39（76.5%） cases in Binet B/C. There were 29 patients（20.9%） in Ann Arbor or Lugano stage I-II and 110 cases（79.1%） in stage III-IV of other subtypes. The complete remission（CR） rate was 43.1%（25/58）， 40.2%（39/97）， 7.1%（1/14）， and overaIl response rate（ORR） was 87.9%（51/58）， 62.9%（61/97）， 28.6%（4/14） in the groups of BTK inhibitors， rituximab-based therapy， and chemotherapy alone. The 3-year OS rate and PFS rate in all patients was 79.2% and 72.4% respectively. The 3-year OS rate of patient with MZL, CLL/SLL, FL,WM was 94.7%, 87.7%, 86.8% and 83.3% respectively, while the 3-year OS rate of MCL was only 40.6%, which was significantly lower than other subtypes. The median OS of patients treated with BTK inhibitors and rituximab-based therapy was 20.5 and 18.5 months respectively， and the 3-year OS rate was 97.4% and 90.7%. However， the median PFS of patients receiving chemotherapy alone was 4 months， and the 1-year OS rate was 52.7%， which was statistically significant compared with the other two groups（P<0.05）. Univarite analysis showed that anemia， elevated lactate dehydrogenase， elevated β2-microglobulin， and splenomegaly were the poor prognostic factors for OS（P<0.05）， elevated lactate dehydrogenase was also poor prognostic factors for PFS（P<0.05）. Multifactor analysis showed that anemia and elevated lactate dehydrogenase were the independent poor prognostic factors for survival（P<0.05）.@*CONCLUSION@#The clinical features of B-CLPD was various， anemia and elevated lactate dehydrogenase are the prognostic factors for poor survival. BTK inhibitors and new immunotherapy can improve the survival and prognosis of patients in the new drug era.

Utility of CD200 expression and CD20 antibody binding capacity in differentiating chronic lymphocytic leukemia from other chronic lymphoproliferative disorders

Background: Chronic lymphoproliferative disorders (CLPDs) are heterogeneous group of disorders with variable clinical presentations and outcomes. Therefore, accurate classification is crucial for treatment planning. At present, flow cytometry immunophenotyping (FCM-IPT) is a useful tool for diagnosing these diseases. However, overlapping immunophenotypes do exist. Recently, differential expression of CD200 and variation in number of CD20 antibody bound per cell (ABC) in different CLPDs has been reported. Materials and Methods: Seventy-seven CLPD cases were analyzed by FCM-IPT for CD200 expression, and Quantibrite bead was used to calculate CD20 ABC. Results: Variability in CD200 expression can help in the differentiation of chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) from other CLPDs. CD200 was brightly expressed in 100% CLL cases, having homogenous bright (2+) intensity. On the contrary, CD200 was uniformly negative in all Mantle cell lymphoma cases except 1, in which the intensity was dim, and the mean fluorescence intensity was significantly lower than CLL. Furthermore, all HCL cases showed bright expression of CD200, thereby making it useful in differentiation from other CLPD with villous lymphocytes. Evaluation of CD20 ABC showed that it differs among various CLPD and was significantly lowest in CLL and highest in HCL both on peripheral blood and bone marrow samples. Conclusion: Our results support the fact that CD200 can be added to routine CLPD panel as it is useful in subcategorizing them. However, inclusion of CD20 ABC to routine panel does not seem plausible but may be done for difficult diagnostic cases or where anti-CD20 therapy is planned.

Case report of adult T-cell lymphoma/leukemia / Relato de caso de linfoma/leucemia de células T do adulto

The adult T-cell leukemia/lymphoma (ATLL) is a rare type of lymphoma caused by human T lymphotropic virus type 1 (HTLV-1). The clinical manifestations include cutaneous lesions, adenopathies, myelopathy/ tropical spastic paraparesis, uveitis, ophthalmological diseases, leukocytosis with lymphocytosis and atypical lymphocytes. The main objective of this study was to report a case of a female patient with ATLL, a farmer with leukocytosis, lymphocytosis, bilateral ocular erythema, cervical lymphadenopathy, in the abdominal visceromegalies and with positive markers for T-cell lymphocytes (CD45, CD2, CD3, CD5, CD4 and CD25). Although ATLL is a rare disease, its delayed diagnosis may lead to serious complications and fatal outcome.

O linfoma/leucemia de células T do adulto (ATLL) é um tipo raro de linfoma causado pelo vírus T-linfotrópico humano tipo I (HTLV-1). O quadro clínico inclui lesões de pele, adenomegalias, mielopatia/paraparesia espástica tropical, uveíte, doença oftalmológica, leucocitose com linfocitose e linfócitos atípicos. O objetivo deste estudo foi relatar o caso de uma paciente com ATLL, agricultora, com leucocitose, linfocitose, eritema ocular bilateral, linfadenopatia cervical, sem visceromegalias abdominais e com marcadores positivos para linfócitos T (CD45, CD2, CD3, CD5, CD4 e CD25). Embora a ATLL seja uma doença rara, a demora no seu diagnóstico pode levar a sérias complicações e ocasionar a morte do paciente.