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Objective: To investigate the clinical, biological and prognostic characteristics of leukemic non-nodal mantle cell lymphoma (nnMCL). Methods: The clinical data of 14 patients with nnMCL and 238 patients with classical mantle cell lymphoma (cMCL) in Blood Diseases Hospital, Chinese Academy of Medical Sciences from November 2000 to October 2020 were retrospectively analyzed. Among the 14 patients with nnMCL, there were 9 males and 5 females, with the age [M ( Q 1 , Q 3 )] of 57.5 (52.3, 67.0) years. Among the 238 patients with cMCL, there were 187 males and 51 females, with the age of 58.0 (51.0, 65.3) years. The clinical and biological characteristics of the two groups were recorded and compared. Follow-up and efficacy evaluation were conducted by re-examination during hospital stay and telephone follow-up and so on. Results: The proportion of CD200 expression in nnMCL patients was 8/14, which was higher than that in cMCL patients [14.6% (19/130)] ( P =0.001). The proportion of CD23 expression in nnMCL patients was 8/14, which was higher than that in cMCL patients [13.5% (23/171)] ( P <0.001). The proportion of CD5 expression in nnMCL patients was 10/14, which was lower than that in cMCL patients [97.4% (184/189)] ( P =0.001). The proportion of CD38 expression in nnMCL patients was 4/14, which was lower than that in cMCL patients [69.6% (112/161)] ( P =0.005). The expression proportion of sex-determining region of Y chromosome-related high-mobility-group box 11 (SOX11) in nnMCL patients was 1/5, which was lower than that in cMCL patients [77.9% (60/77)] ( P =0.014). The proportion of immunoglobulin heavy chain variable region (IGHV) mutations in nnMCL patients was 11/11, which was higher than that in cMCL patients [26.0% (13/50)] ( P <0.001). As of April 11, 2021, the follow-up time for nnMCL and cMCL patients was 31 (8-89) months and 48 (0-195) months, respectively. Among the 14 nnMCL patients, 6 patients were still under observation, and 8 patients were treated. The overall response rate (ORR) was 8/8, including 4 patients with complete remission and 4 patients with partial response. The median overall survival and median progression-free survival were not reached in nnMCL patients. In the cMCL group, 50.0% (112/224) patients achieved a complete response, 24.6% (55/224) patients achieved a partial response, and ORR was 74.6% (167/224). There was no statistically significant difference in ORR between the two groups ( P =0.205). Conclusions: nnMCL patients have an indolent progression, with higher expression rates of CD23 and CD200 and lower expression rates of SOX11, CD5 and CD38. Most patients have IGHV mutations, with a relatively good prognosis, and"watch and wait"approach is an optional treatment.

Objective: To explore the relationship between the findings of whole body diffusion weighted imaging (WBDWI) and the clinical result in patients with multiple myeloma. Methods: A total of 43 cases of multiple myeloma patients were retrospectively collected from May 2015 to May 2017 in Tianjin First Central Hospital.Twenty-nine cases were male and 14 were female. The median age was 54 years old with a range from 36 to 73 years old. The patients were divided into two groups with and without abnormal findings pending on whole body diffusion weighted imaging. The clinical data and the ADC value were compared between the two groups, as well as comparison in patients with abnormal findings between pre-and post-treatment. Results: In 43 patients, normal findings on WBDWI were found in 10 cases, 7 males, 3 females, age (59±9) years old, and abnormal findings in 33 cases, 22 males, 11 females, age (57±10) years old.No statistical differences of age and gender were found between two groups ( P> 0.05) .The ratio of plasma cells and the proportion of β(2) microspheres in patients with abnormal WBDWI (50.0% (14.0%, 78.0%) , 4.8 (2.7, 7.7) mg/L)were significantly higher than those in the normal group(5.0% (2.5%, 15.0%) , 2.4 (2.0, 3.7) mg/L) ( P< 0.05).ADC value in different body parts of abnormal group including costal ((0.66±0.15)×10(-3) mm(2)/s), sternal bone((0.71±0.20)×10(-3) mm(2)/s), clavicles((0.67±0.17)×10(-3) mm(2)/s), thoracic vertebra((0.63±0.17)×10(-3) mm(2)/s), lumber vertebra((0.69±0.20)×10(-3) mm(2)/s), pelvic((0.83±0.36)×10(-3) mm(2)/s), proximal humerus((0.76±0.13)×10(-3) mm(2)/s), proximal femur((0.64±0.17)×10(-3) mm(2)/s), shaft of femur((0.70±0.22)×10(-3) mm(2)/s), proximal tibia((0.97±0.18)×10(-3) mm(2)/s), shaft of tibia((0.83±0.18)×10(-3) mm(2)/s) which were significantly higher than those of normal group (all P< 0.05). The albumin concentration of the patients after treatment was significantly higher than those before treatment ( P< 0.05). Conclusion: Different imaging findings on WBDWI can reflect clinical different result in patients with multiple myeloma, and when WBDWI is normal, the clinical symptoms are mild. When abnormal findings detected on WBDWI, the clinical symptoms are still severe although albumin concentration increased after treatment.

Objective: To explore the clinical and laboratory characteristics and survival of Chinese patients with hairy cell leukemia (HCL)., Methods: A total of 30 HCL patients from August 1990 to March 2012 were retrospectively analyzed., Results: There were 22 cases with classical HCL (HCL-C) and 8 with variant HCL (HCL-V). Splenomegaly was the most common physical finding. Leukocytosis was found in all cases of HCL-V. But pancytopenia only accounted for 36.4% (8/22) in HCL-C. And 3/5 of HCL-V had abnormal chromosome karyotypes. Ribosomal-lamellae complexes (RLC) were found only in about 3/12 of HCL cases. Chemotherapy regimens including purine nucleoside analogues achieved a better complete remission (CR) rate than other regimens (3/4 vs 1/18, P = 0.012) in HCL-C. The median follow-up period was 27 (1 - 142) months. There was no follow-up loss. Eleven cases progressed and 6 died. The median overall survival (OS) was not reached. And the 1, 3, 6-year OS rates were 84%, 78% and 58% respectively. The median progression-free survival (PFS) was (63 ± 24) months and the 1, 2, 5-year PFS rates were 86%, 72% and 44% respectively. The median PFS of HCL-V was significant shorter than HCL-C ((23 ± 3) vs (78 ± 12) months, P = 0.014). In HCL-C group, fever (P = 0.038) and anemia (P = 0.000) at diagnosis were poor prognostic factors. But purine nucleoside analogues made no significant difference in PFS., Conclusions: Pancytopenia is infrequent in Chinese HCL patients. And classical RLC is rare under electron microscope. Purine nucleoside analogues may achieve a better CR rate, but fail to improve PFS rate. As compared with HCL-C, HCL-V is common with genetic abnormalities and has a worse prognosis with a shorter PFS.

Objective: To summarize the clinical characteristics of Richter syndrome and explore the methods of successful treatment and timely diagnosis., Methods: Five patients with Richter syndrome in the last three years (from January 2009 to December 2011) were analyzed retrospectively at our hospital, including their clinical features and therapy before and after transformation., Results: There were 4 males and 1 female with a median age on a diagnosis of chronic lymphocytic leukemia (CLL) at 47 (44 - 68) years. The median duration from a diagnosis of CLL to transformation was 52 (5 - 90) months. As for cytogenetic abnormalities, 3/4 patients had 17p deletion by fluorescence in situ hybridization (FISH). The clinical manifestations on transformation included regional enlargement of lymph node (n = 2) and systemic enlargement of lymph nodes (n = 3). All diagnoses were confirmed by lymph node biopsy and all transformed into diffuse large B cell lymphoma (classical transformation). The subgroups were germinal center B-cell like (GCB) (n = 3) and non-GCB (n = 1). After transformation, one patient underwent sibling allo-stem cell transplantation and survived 24 months until April 2012. Another patient with auto-stem cell transplantation relapsed and died 12 months later. One patient lost the treatment opportunity due to worsening condition. Another 2 patients gained partial remission after therapy and survived 20 and 8 months respectively., Conclusions: Richter syndrome may occur during a late stage of CLL. Such a high-risk cytogenetic abnormality as del17p may be correlated with transformation. Early identification and optimal therapy may extend the survival of Richter syndrome. Allo-stem cell transplantation remains a curable option.

Objective: To detect the expression of microRNA-223 and analyze its clinical value in B lymphoproliferative disorders., Methods: Peripheral blood samples (n = 78) and bone marrow samples (n = 9) were collected from patients with chronic lymphocytic leukemia (CLL, n = 53), mantle cell lymphoma (MCL, n = 13), splenic marginal zone lymphoma (SMZL, n = 9) and healthy donors (n = 12) at our hospital from 2003 to 2010. Mononuclear cells were isolated and B cells purified with a CD19(+) magnetic-bead system. Total RNA was extracted from purified CD19(+) cells and the expression of microRNA-223 measured by TaqMan microRNA quantitative polymerase chain reaction (PCR). The clinical data of these patients were collected and their outcomes analyzed with SPSS 16.0 software., Results: (1) The levels of microRNA-223 in CLL, MCL and SMZL were 4.58 ± 0.62, 4.03 ± 0.54 and 4.63 ± 0.57 respectively. And they were significantly lower than that in normal B cells (5.69 ± 0.60, P < 0.01). The expression of microRNA-223 decreased significantly in MCL versus CLL and SMZL (P < 0.05). There was no statistical difference between CLL and SMZL (P > 0.05). (2) The down-regulation of microRNA-223 was associated with disease aggressiveness in CLL. Patients with unmutated immunoglobulin heavy chain variable region (IgVH) expressed significantly a lower level of microRNA-223 (4.05 ± 0.69 vs 4.67 ± 0.51, P = 0.003). In 13q-negative patients, the expression of microRNA-223 decreased more significantly than that in 13q-positive patients (4.25 ± 0.67 vs 4.76 ± 0.45, P = 0.044). (3) Using receiver operating characteristic (ROC) curve analysis, the microRNA-223 cutoffs were defined according to the IgVH mutational status. The patients were divided into the positive and negative subgroups. The median progression-free survival (PFS) of microRNA-223 positive patient subgroup was 48 months. It was significantly longer than the negative subgroup (P = 0.001). In the microRNA-223 positive subgroup, no patient died at the end of follow-up., Conclusions: MicroRNA-223 may play an important role in the pathogenesis of B lymphoproliferative disorders. The down-regulation of microRNA-223 is associated with disease aggressiveness and poor prognostic factors in CLL. It may become a new reliable prognostic predictor.

Objective: To summarize the clinical significance and 13q- characters of 100 multiple myeloma (MM) patients detected by interphase fluorescence in situ hybridization (i-FISH)., Methods: Specimens of bone marrow were collected from 100 patients with MM who visited the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences in Tianjin, China. Chromosome R-banding analysis and i-FISH were conducted., Results: (1) i-FISH was used to investigate 100 patients with MM, whose median age was 56 years and 19 (19.0%) cases showed chromosome 13q deletions/monosony13 (Delta13); conventional cytogenetics (CC) revealed informative MM karyotypes in 24 patients (24.0%),with Delta13 in 10 (10.0%) of them. Detection rate of the two methods had no significant difference (P = 0.053), but in newly diagnosed patients i-FISH was much more sensitive than CC test (Detection rate, 25.3% vs 9.3%, P = 0.008). (2) Among the whole cases, 93 of them had complete follow-up information. The overall survival (OS) of the 93 patients was 41 (1-69) months. Univariate analysis showed that the positive rate of Delta13 detected by i-FISH >50%, clonal chromosome 13 abnormality (C13A), nonhyperdiploidy, Delta13 detected by both CC and i-FISH, beta2-MG > or = 3.5 mg/L were associated with significantly shorter OS. Multivariate analysis indicated that the positive rate of Delta13 detected by i-FISH > 50% and beta2-MG > or = 3.5 mg/L were the independent unfavorable factors. (3) According to the two independent unfavorable factors mentioned above, we divided the 93 patients into three groups: low-risk, standard-risk and high-risk. And there were significant differences of OS among the 3 groups (P < 0.05)., Conclusion: (1) FISH studies demonstrate a high sensitivity at detecting chromosome 13 abnormality and should be used in the routine evaluation of MM. (2) The positive rate of Delta13 detected by i-FISH > 50% and beta2-MG > or = 3.5 mg/L were the independent adverse prognostic factors.

Objective: To investigate the difference of clinical characteristics and outcomes between different isoforms of BCR/ABL in adults with Philadelphia-positive acute lymphoblastic leukemia (ALL)., Methods: The data of 106 adults with Ph+ ALL diagnosed in our hospital from January 1, 1996 to December 31, 2007 were reviewed. The difference of clinical characteristics between different subgroups of BCR/ABL was compared and their relation with outcomes was studied., Results: The median age of the 106 patients was 34 years and the median white blood cell count at baseline was 28.5 x 10(9)/L. Comparative analysis demonstrated that patients in p210 group had an older age, higher blood platelet count (BPC) and more frequent occurrence of splenomegaly. Referring to the outcomes, the complete remission (CR) rate of the two groups were 92.2% and 93.9%, respectively. The median overall survival (OS) and relapse free survival (RFS) in p190 group were 13 months and 10 months, the 1, 3-year estimated OS were (54.7 +/- 6.7)% and (5.5 +/- 5.2)%, and the 1, 3-year estimated RFS were (40.2 +/- 6.8)% and (7.8 +/- 6.7)%, while in p210 group, the median OS and RFS were 15 months and 10 months, respectively, the 1, 3-year estimated OS were (65.8 +/- 8.9)% and (14.5 +/- 7.4)%, and the 1, 3-year estimated RFS were (48.3 +/- 9.4)% and (12.9 +/- 7.7)%. All of the above data had no statistic significance between the two groups., Conclusion: Majority of the adults with Ph+ ALL is p190 positive and patients with p210 have older age, higher BPC and more frequent occurrence of splenomegaly, while there is no significant difference between p190 group and p210 group in CR rate, RFS and OS.

Objective: To identify the clinical and pathological features of blastic plasmacytoid dendritic cell neoplasm (BPDC)., Methods: The characteristics of BPDC hematodermic neoplasm were discussed with a report of two new cases and review the literatures., Results: Both patients presented with skin nodules and the tumors were CD(4)(+) and CD(56)(+). Lineage specific markers for B- and T-cell were negative and the tumors did not express myeloperoxidase. Systemic chemotherapy resulted in complete remission, but the disease relapsed quickly and were unresponsive to further chemotherapy. The patients died 26 months and 11 months respectively after diagnosis., Conclusion: BPDC hematodermic neoplasm is a rare subtype of lymphoma with distinct clinicopathologic and immunophenotypic features. The disease often has a fulminant course with a poor prognosis. More recent studies suggest that there is a derivation from a plasmacytoid dendritic cell precursor.

Objective: To investigate the effects of different treatment protocols on non-Hodgkin's lymphoma (NHL) with bone marrow involvement (BMI)., Methods: The clinical data of 148 patients of NHL with BMI, 108 undergoing CHOP regimen, 51 undergoing dose-intensive CHOP regimens, 13 undergoing CHOP regimen + rituximab, and 27 undergoing hemopoietic stem cell transplantation (HSCT) after CHOP protocol with remission. The curative effects were retrospectively analyzed., Results: (1) The response rate (RR) for the whole group was 80.4%, with a complete remission (CR) rate of 60.7%. The response rate of the patients treated with the dose-intensive regimens (dose-intensive group) was 84.3%, significantly higher than that of the patients treated with standard CHOP regimen (64.1%, P < 0.05). (2) The 3- and 5-year overall survival (OS) rates and progress-free survival (PFS) of the B-cell NHL patients who underwent HSCT + Rituximab treatment (Rituximab combined with chemotherapy) were significantly higher than those of the dose-intensive group and CHOP group, and the 3- and 5-year OS rates and PFS of the dose-intensive group were all significantly higher than those of the CHOP group (all P < 0.05). (3) The 3- and 5-year OS rates, and PFS of the T-cell NHL patients who underwent HSCT group and dose-intensive CHOP were all significantly higher than those patients who underwent CHOP regimen (all P < 0.05)., Conclusion: Superior survival may benefit from Dose-intensive regimens and HSCT, may be good choices for the patients with NHLBMI, a group of heterogeneous malignancies with poor prognosis. And combined chemotherapy with Rituximab treatment remarkably raises the effect for CD20(+) B-cell NHLBMI.

Objective: To summarize the cytogenetic characteristics of patients with multiple myeloma (MM) in China and clinical significance thereof., Methods: Specimens of bone marrow were collected from 100 patients with MM who visited the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences in Tianjin, China. Chromosome banding analysis and fluorescence in situ hybridization (FISH) were conducted., Results: (1) Clonal chromosome aberrations (CA) were detected in 16 of 93 patients (17.2%) by conventional cytogenetics (CC), and when subclonal CA was included CA were detected in 37.0% of the patients. Numerical aberration analysis showed that the most common trisomies were 11, 21, 3, 6, and 12, and the most common monosomies were 16, 13, 8, 22, and 11. Structural aberration analysis showed that the most frequently involved arms were 14q, 8q, 1q, 6q, 11q, 12p, and 1p, and the most frequently involved breakpoints were 14q32, 8q22 - 24, 11q13 - 14, 6p21, 1q10 - 11, and 12p12 - 13. Ploidy level was arranged in decreasing order as: hypodiploidy (46.1%), pseudodiploid (38.5%), hyperdiploidy (18.8%), and tri-/tetraploidy (15.4%). Chromosome 13 abnormality (C13A) was detected in 7 of the 100 patients (7%). The C13A prevalence rates by CC and interphase FISH were 6.0% and 33.3% respectively. Translocations involving chromosome 14 and/or 14q32 aberrations were detected in 6 patients (6.0%). (2) Univariate analysis showed that C13A, nonhyperdiploidy, and clonal CA were associated with significantly shorter overall survival (OS) and time to progression (TTP). Multivariate analysis showed that C13A was the only independent unfavorable factor., Conclusion: The concrete CA of the patients with MM in China are comparable to those from Western countries, among which C13A, nonhyperdiploidy, hyperdiploidy, and translocations involving immunoglobulin heavy chain locus (IgH) are recurrent. C13A, nonhyperdiploidy, and clonal CA are associated with shorter OS and time-to-progression, and C13A is the only independent adverse prognostic factor.