Clinicopathologic features and differential diagnosis of splenic B-cell marginal zone lymphoma involving bone marrow / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of splenic B-cell marginal zone lymphoma (SMZL) involving bone marrow.</p><p><b>METHODS</b>The clinical and pathologic features of 22 patients with SMZL were retrospectively studied. Immunophenotypic analysis was carried out by flow cytometry and immunohistochemistry. Immunoglobulin heavy chain rearrangement study was performed using polymerase chain reaction-based method.</p><p><b>RESULTS</b>Villous lymphocytes were found in peripheral blood smears of 11/18 of the patients. In bone marrow aspirates, lymphocytosis (> 20%) was demonstrated in 15 cases (15/18) and villous lymphocytes in 6 cases (6/18). Flow cytometry showed CD19(+) CD20(+) FMC7(+) CD22(+) CD10(-) CD2(-) CD3(-) CD7(-) in 18 cases. Bone marrow biopsies of all the 22 patients revealed various degrees and patterns of neoplastic infiltration, as follows: mild (4 cases, 18.2%), moderate (11 cases, 50.0%) or severe (7 cases, 31.8%); intrasinusoidal (16 cases, 72.7%), interstitial (14 cases, 63.6%), nodular (11 cases, 50.0%) or diffuse (1 case, 4.5%). Reactive germinal center formation (CD23(+) bcl-2(-)) was found in 2 cases (91.0%). Immunohistochemical study showed the following results: CD20(+) PAX5(+) CD3(-) CD5(-) CD10(-) cyclin D1(-) CD23(-) CD43(-) Annexin A1(-) CD11C(-) CD25(-) in all the 22 cases, CD38(+) in 2 cases (9.1%) and CD138(+) in 2 cases (9.1%).</p><p><b>CONCLUSIONS</b>Different and overlapping patterns of bone marrow involvement are observed in SMZL. As the histologic and immunophenotypic features are not specific to SMZL, distinction from other types of mature B-cell lymphomas is necessary.</p>

Analysis of clinical features and prognosis of primary myelodysplastic syndromes with myelofibrosis patients / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze the clinical features and prognosis of the primary myelodysplastic syndrome with myelofibrosis (MDS-MF) patients and to improve the cognition of MDS-MF.</p><p><b>METHODS</b>Four hundred and sixty-six primary MDS patients with bone marrow (BM) biopsy were divided into two groups according to whether BM associated with fibrosis, the clinical features and prognosis of the two groups were analyzed retrospectively.</p><p><b>RESULTS</b>167 (35.8%) MDS cases revealed myelofibrosis, of which MF-1 123 cases (26.4%), MF-2 40 cases (8.6%), MF-3 4 cases (0.9%). The proportion of hepatosplenomegaly in MDS-MF group was significantly higher than in MDS without MF group, the difference had statistical significance (P = 0.031). The proliferation of BM biopsy in MDS-MF group was significantly more active than in MDS without MF group. The number of blasts, megakaryocytes and abnormal megakaryocytes in MDS-MF group were significantly higher than in MDS without MF group, the differences had statistical significance (P < 0.05). Among the 345 patients who had available results of cytogenetic analysis, 121 cases were MDS-MF patients, the proportion of middle and high-risk prognostic group according to IPSS karyotype prognosis groups in MDS-MF group were significantly higher than in MDS without MF group, the differences had statistical significance (P = 0.047). The median survival was 17 (1 - 60) months in MDS-MF group, and was 32 (1 - 62) months in MDS without MF group. The difference had statistical significance (P = 0.001). Myelofibrosis had independent prognostic significance by multi-variable analysis (P = 0.019).</p><p><b>CONCLUSION</b>The myelofibrosis in MDS is main the proliferation of reticular fiber. The proliferation of reticular fiber is closely related with the number of blast cells, the proliferation and developmental abnormalities of megakaryocytes and the karyotype. The prognosis of MDS-MF patients is poor.</p>

Hematopathologic features of T-cell large granular lymphocytic leukemia / 中华病理学杂志

<p><b>OBJECTIVE</b>To explore the hematopathologic features of T-cell large granular lymphocytic leukemia (T-LGLL).</p><p><b>METHODS</b>A retrospective analysis of the clinical presentation, bone marrow morphology, immunophenotyping and T-cell receptor gene rearrangement status were performed in 19 patients with T-LGLL.</p><p><b>RESULTS</b>Of 19 patients, the most frequent hematological abnormalities were anemia and neutropenia (16/19 and 17/19 patients, respectively). Large granular lymphocytes (LGLs) were observed in 17 of 19 peripheral blood smears and 15 of 19 bone marrow aspirate specimens. Lymphocytosis (> 0.2) was present in 17 of 19 patients in their bone marrow aspirate specimens. Bone marrow biopsy specimens revealed lymphocytosis in 16 cases, with a mild to moderate increase of lymphocytes observed in 12 cases (12/16). The pattern of lymphoid distribution was interstitial in bone marrow sections. Intravascular distribution was seen in 8 cases. Lymphoid nodules were present in 4 cases. Flow cytometery showed an immunophenotype of CD3(+) CD4(-) CD8(+) CD56(-) CD57(+) of the tumor cells in 13 cases. Of the other 6 cases, the immunophenotypes included CD8(-) (1 case), CD56(+) (2 cases) and CD57(-) (3 cases). Immunohistochemistry showed CD3+ (10/10), CD57+ (3/3), CD8+ (6/7), TIA-1+ (6/7), granzyme B+ (4/7), perforin + (1/7), CD4- (4/4) and CD56- (9/9). Clonal T-cell receptor γ gene rearrangement by PCR was detected in 12 cases (12/17).</p><p><b>CONCLUSIONS</b>Hematopathologic features of most T-LGLL are distinct. Morphologic, immunophenotypic and molecular analysis of both peripheral blood and bone marrow specimens are essential and complementary in the diagnosis and differential diagnosis of T-LGLL.</p>

Clinical observation of treating 62 patients with severe aplastic anemia failing in immunosuppressive therapy by integrative medicine / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To explore treatment methods for patients with severe aplastic anemia (SAA) failing in immunosuppressive therapy (IST).</p><p><b>METHODS</b>Totally 62 SAA patients failing in IST were treated by integrative medicine (IM). The treatment course was divided into three stages: the critical emergency stage, the improvement stage, and the recovery stage. In the critical emergency stage, patients were treated with Lingyang Yigui Decoction (LYD, consisting of 1.2 g antelope horn, 6 g coptis chinensis, 12 g stir-baked Fructus Gardeniae, 30 g rehmannia rhizoma, 50 g lalang grass rhizome, 9 g amur corktree bark, 12 g Cortex Moutan, 9 g ass-hide gelatin, 30 g red date, 6 g prepared licorice root, etc.) and Erzhi Busui Decoction (EBD, consisting of 120 g glossy privet fruit, 100 g eclipta prostrata, 24 g prepared Gold Theragran, 12 g fructus lycii, 90 g rehmannia rhizoma, 60 g astragalus, 9 g Angelica sinensis, 9 g ass-hide gelatin, 30 g honeysuckle flower, 12 g lotus plumule, and so on) alternatively, one dose daily, decocted twice, taken in two portions. Meanwhile, 50 mg Testosterone Propionate was intramuscularly injected every other day to the improvement stage. Those with fever were treated with LYD by adding 60 g gypsum, 60 g common anemarrhena, 30 g dandelion, 30 g bittersweet herb, 30 g blackend swallowwort root and rhizome, 15 g hemsley rockvine root tuber, and so on. In the improvement stage patients were treated with Jixueteng Compound (Jixueteng Zhengyang Decoction was administered to those of Shen-yang deficiency syndrome: consisting of 100 g spatholobus suberectus, 60 g astragalus, 3 g red ginseng, 12 g psoralea corylifolia, 18 g dodder seed, 12 g angelica, 18 g Herba Epimedii, 6 g common fenugreek seed, 24 g Gold Theragran, 30 g glossy privet fruit, 30 g eclipta prostrata, 6 g dried human placenta, and so on). Meanwhile, 50 mg Testosterone Propionate was intramuscularly injected every other day. Jixueteng Yijing Decoction was administered to those of Shen-yin deficiency syndrome: consisting of 100 g glossy privet fruit, 100 g eclipta prostrata, 90 g rehmannia rhizoma, 30 g spatholobus suberectus, 12 g dodder seed, 6 g psoralea corylifolia, 30 g prepared Gold Theragran, 9 g ass-hide gelatin, 9 g fructus lycii, 24 g Salvia miltiorrhiza, 30 g astragalus, 6 g angelica, and so on), one dose daily, decocted twice, taken in two portions. The treatment lasted to the recovery stage. The medication was gradually reduced to the follow-ups of drug discontinuance. Results After 6 -57 months of treatment, 12 patients (accounting for 19.4%) were basically cured, 14 (22.6%) relieved, 8 (12. 9%) markedly improved, 28 (45.2%) ineffectively, with the total effective rate of 54. 8%. Totally 23 patients had the body temperature ranging 37.6-38.5 degrees C at the first visit to our hospital. They took 2 h- 6 days to have pyretolysis ( <37.5 degrees C) after treatment. Twenty patients with body temperature higher than 38.5 degrees C took 4 h - 5 days to have pyretolysis after treatment. Totally 26 patients suffering from IST induced abnormalities of liver and renal functions (ALT, AST, BUN, and Cr) at the first visit were treated by IM for 2 months. They were restored to the normal levels in 25 cases.</p><p><b>CONCLUSIONS</b>The treatment of SAA failing in IST had its specificity. The staging targeted treatment is in line with its pathophysiology. The key points for its treatment might be lie in the improvement and protection of hematopoietic microenvironment of bone marrows. The antisepsis and anti-inflammation of Chinese herbs hindered its aggravating tendency.</p>

Clinicopathologic features of aggressive natural killer cell leukemia / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of aggressive natural killer cell leukemia (ANKL).</p><p><b>METHODS</b>The clinical and pathologic features were analyzed in 10 patients with ANKL. The complete blood count, peripheral blood smears, bone marrow aspirates and bone marrow biopsies were studied. Immunophenotypic analysis was carried out by flow cytometry and immunohistochemistry. T-cell receptor (TCR) γ gene rearrangement was studied by PCR method.</p><p><b>RESULTS</b>The most frequent hematologic abnormalities observed were anemia (7 cases) and thrombocytopenia (9 cases). Large granular lymphocytes were found on peripheral blood smears of 6 patients. In bone marrow aspirates, lymphocytosis (> 20.0%) was demonstrated in 8 cases and large granular lymphocytes in 6 cases. Bone marrow biopsies revealed various degrees of neoplastic infiltration, as follows: mild (5 cases), moderate (3 cases) and severe (2 cases). The neoplastic cells were mainly interstitial in distribution in 8 cases and diffuse in 2 cases. Hemophagocytosis was observed in 4 cases. Flow cytometry showed CD2+ sCD3- CD4- CD56+ CD57- in all cases, CD7+ in 9 cases, CD16+ in 5 cases, CD8+ in 4 cases and CD5+ in 1 case. Immunohistochemistry performed in 8 cases showed the following results: cCD3+ in 4 cases, CD56+ in 6 cases, TIA-1+ in 6 cases, granzyme B+ in 4 cases and perforin+ in 2 cases. PCR study revealed germline TCRγ gene configuration in all cases.</p><p><b>CONCLUSIONS</b>ANKL is a highly aggressive NK cell-derived lymphoid neoplasm. Comprehensive morphologic, immunophenotypic and molecular analysis are essential in arriving at a correct diagnosis. ANKL needs to be distinguished from other types of NK-cell and T-cell lymphomas.</p>

Clinicopathologic study of 15 splenectomy specimens of patients with hairy cell leukemia / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features, diagnosis, differential diagnosis and the prognosis of hairy cell leukemia (HCL).</p><p><b>METHODS</b>Fifteen splenectomy specimens of HCL patients were investigated retrospectively using HE and immunohistochemistry in correlation with the follow-up information.</p><p><b>RESULTS</b>(1) The male to female ratio was 2.75:1, age ranged from 36 to 68 years with a median of 47 years. The most consistent clinical feature at presentation was marked splenomegaly (100%). Other symptoms included anemia (80.0%), thrombocytopenia (60.0%), leucocytosis (53.3%), pancytopenia (20.0%) and the absence of B-symptom. (2) The proportion of hairy cells was (14.6 +/- 7.2)% in periphery blood and (47.3 +/- 23.8)% in bone marrow. The positive rate of TRAP assay was 62.5% in bone marrow; 85.7% for TPA test and the detection rate for RLC was 25% by transmission electric microscopy. The frequency of bone marrow involvement was 100%. (3) The average weight of 15 spleens was (3012 +/- 1974) g. The size of 6 spleens ranged from 16 cm x 10 cm x 5 cm to 32 cm x 20 cm x 14 cm. The white pulp of spleen showed a characteristic atrophy feature or even absent due to leukemic infiltration, predominantly involving the red pulp with some sinusoidal pattern. "Blood pool" change was an infrequent feature (3/15 cases). The nuclei of leukemic cells were round (13 cases) or bean-shaped (2 cases), nucleoli inconspicuous or disappeared. The abundant cytoplasm and prominent cell border resulted in a "fried egg" appearance. By immunohistochemistry, leukemic cells were positive for CD45RA, CD20, PAX-5, CD25, CD11c, Annexin A1 and cyclinD1, but negative for CD3 and CD43. (4) 13 cases (86.7%) have been followed-up and all are alive. Among them, 9 cases are living well more than 5 years and 7 more than 10 years.</p><p><b>CONCLUSIONS</b>Splenomegaly is frequently the first manifestation of patients with HCL and occurred predominantly in the middle to elderly adults. Definite diagnosis of HCL requires a combined histological and immunohistochemical assessment of the splenectomy specimen, bone marrow biopsy and aspirate.</p>

Clinical significance of ZAP-70 protein expression in chronic lymphocytic leukemia/small lymphocytic lymphoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and prognostic significance of ZAP-70 protein expression in chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL).</p><p><b>METHODS</b>The histologic features of 52 cases of CLL/SLL with lymph node and/or bone marrow biopsies performed were retrospectively reviewed. Immunohistochemical study using EliVision for ZAP-70 protein was adopted.</p><p><b>RESULTS</b>The lymph nodes of the 12 cases studied showed effacement of the nodal architecture and was replaced by a monotonous infiltration of small lymphoid cells. Among them, proliferation centers were identified in 6 cases. Similar morphologic pattern was seen in the 40 bone marrow biopsy samples, but no proliferation center formation obtained. The infiltration pattern of tumor cells in the bone marrow were further subdivided into nodular (n = 9), interstitial (n = 3), mixed (n = 9) and diffuse types (n = 19). There was no significant difference found on survival rates between the diffuse infiltration and non-diffuse infiltration groups (Fisher's exact test, P = 0.199). ZAP-70 protein was mainly located in the cytoplasm and nuclei of lymphoma cells. There were 21 cases (40.4%) positive for ZAP-70 and among them, 11 died of this disease or the related infections. On the other hand, ZAP-70 was negative in 31 cases (59.6%) and only 4 of them died of this disease or related infections. The overall survival in ZAP-70-negative group was higher than that of the ZAP-70-positive group (59 months versus 39 months, chi(2) = 6.991, P = 0.008). Follow-up information was available in 51 patients. Among the 21 dead cases, 15 died of CLL/SLL or the related infection.</p><p><b>CONCLUSION</b>A positive expression of ZAP-70 protein in CLL/SLL suggests a poor prognosis.</p>

Clinicopathologic features of peripheral T-cell lymphoma, unspecified with follicular pattern / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of peripheral T-cell lymphoma, unspecified (PTL-U) with follicular pattern.</p><p><b>METHODS</b>The clinical data, hematoxylin and eosin-stained sections of lymph node biopsies and follow-up data of 18 cases of PTL-U associated with follicular growth pattern were reviewed and studied. Eight cases of reactive lymphoid hyperplasia were used as controls. Semi-quantitative observation by retiform micrometer rule was carried out. Immunohistochemical study was also performed in all cases. T-cell receptor and immunoglobulin heavy chain gene rearrangement studies were conducted by polymerase chain reaction-based method.</p><p><b>RESULTS</b>The median age of the patients was 53 years. The male-to-female ratio was 1.57:1 in lymphoma group. All of the lymphoma patients presented with superficial lymphadenopathy, with (8/18) or without B symptoms. Histologically, the lymphoma was characterized by follicles of various sizes and shapes. The T zones were expanded by medium-sized lymphoma cells which contained clear cytoplasm and irregular nuclei. Mitotic figures were commonly identified. Immunohistochemical study confirmed that the lymphoma cells were of T-lineage. The proliferative index, as highlighted by Ki-67, was higher [average = (38.24 +/- 13.42)%/mm2] than that in the control group. T-cell receptor gene rearrangement was demonstrated in 71.4% (10/14) of the lymphoma cases.</p><p><b>CONCLUSIONS</b>A definitive diagnosis of PTL-U with follicular pattern can be made on the basis of morphologic examination, immunohistochemical assessment and clinical features. Cases with atypical features can further be delineated by molecular analysis. Long-term follow up of these patients is prudent.</p>

Clinicopathologic studies of 13 cases of chronic eosinophilic leukemia / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the role of bone marrow biopsy (BMB) in diagnosis and differential diagnosis for chronic eosinophilic leukemia (CEL).</p><p><b>METHODS</b>Clinical and pathological features of thirteen CEL patients were analyzed retrospective. Routine histologic examination was performed on H-G-E, reticulin fiber and toluidine blue stained sections of plastic material emdedded samples of bone marrow biopsies.</p><p><b>RESULTS</b>(1)The male-to-female ratio was 12:1. The median age was 40 (23-67) years old. They presented as fever, anemia, hemorrhage and so on. Most of organs and tissues were also be involved. (2) Peripheral blood counts characterized by eosinophilia (18.1 +/-16.2) x 10(9)/L, (3) BMB showed eosinophils were predominant components, others such as neutrophils, erythrocytes, megakaryocytes were decrease. Degree of reticular fiber was from (1+) to (3+). (4) Follow-up information was available in only 4 patients, whose conditions were stable.</p><p><b>CONCLUSION</b>Combine with the clinical manifestations of CEL patients, it is important in diagnosis and differential diagnosis for CEL by observing the histomorphology features of bone marrow biopsy carefully.</p>

Development of Sweet syndrome in an acute promyelocyte leukemia patient during treatment with all-trans retinoic acid--case report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To identify the side effect of all-trans retinoic acid (ATRA), and improve early therapeutic response in patients with acute promyelocytic leukemia (APL).</p><p><b>METHOD</b>The first case of Sweet's syndrome (SS) developed in a APL patient treated with ATRA was reported in mainland of China, and reviewed correlative literature.</p><p><b>RESULTS</b>Only 14 cases of SS associated with ATRA therapy in APL have been reported in the literature, including the present case. The median age was 49.5 years (9 -84) and 10 were women and 4 men. Of them, SS was restricted to the skin in 10 case, the other 4 muscle, fascia, kidney, and lung were involved. SS appeared after a median of 18 days of ATRA therapy (6 - 34 days). The median WBC count was 7.05 (0.80 - 23.00) x 10(9)/L. Four patients continued with the ATRA therapy without interruption, 13 patients treated with steroids and 12 responded. One patient improved without any treatment. Two cases of SS developed retinoic acid syndromes after ATRA therapy.</p><p><b>CONCLUSION</b>Sweet's syndrome is a rare adverse effect of ATRA, and has similar features with inflammatory or infective dermatosis. The corticosteroids treatment could improve the systemic and cutaneous symptoms. When ATRA therapy was restarted after SS subsided, no recurrence of rashes was observed.</p>

Bone marrow microvessel density and vascular endothelial growth factor expression in patients with aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To study the bone marrow microvessel density (MVD) and vascular endothelial growth factor (VEGF) expression and their clinical significance in patients with aplastic anemia (AA).</p><p><b>METHODS</b>Bone marrow biopsies in 51 newly diagnosed patients with AA were evaluated the MVD and VEGF expression by immunostaining with anti-factor VIII related antigen and VEGF monoclonal antibodies at regular time points after immunosuppressive therapy (IT).</p><p><b>RESULTS</b>The mean bone marrow MVD in AA group was 5.5 +/- 3.5, being significantly lower than that in normal control group (8.7 +/- 3.4, P < 0.05). MVDs of SAA and NSAA patients were 7.4 +/- 2.9 and 4.3 +/- 3.4, respectively, being significantly different (P < 0.01). The VEGF expression in AA group was significantly lower than that in control group [(6.7 +/- 8.4)% vs (14.7 +/- 6.1)%, P < 0.01], but there was no difference between SAA and NSAA. Bone marrow MVD and VEGF were significantly increased after IT in 22 responded AA patients.</p><p><b>CONCLUSION</b>Bone marrow MVD and VEGF expression are low in AA patients which may be one of pathophysiologic mechanisms of bone marrow failure in AA. Proangiogenic and ameliorating microcirculation agents together with IT might accelerate the recovery of hematopoiesis in AA patients.</p>

Study of 161 chronic idiopathic myelofibrosis patients for clinicopathological staging / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the significance of clinicopathological stage of chronic idiopathic myelofibrosis (CIMF) in WHO classification of 2001.</p><p><b>METHODS</b>Histopathological analysis of bone marrow biopsy plastic-embedded sections stained with H-G-E and Gomori's stains and clinical features of 113 cases previously diagnosed as primary myelofibrosis (PMF) and 48 cases MPD-U (total of 161 cases which including male 79 and female 82) were studied retrospectively.</p><p><b>RESULTS</b>There was no significant differences on the clinical features among the cellular phase, collagen fiber phase, sclerotic phase and osteomyelosclerosis of 113 previously diagnosed patients. According to WHO classification 2001 of CIMF, previously diagnosis in 48 cases with MPD-U was WHO pre-CIMF, and in 113 cases with PMF was WHO CIMF-Fs. There were significant differences between of WHO pre-CIMF and WHO CIMF-Fs about clinicopathological features except age. The percentage of immature granulocytes, normoblasts, lymphocytes in peripheral blood, the size of hepatosplenomegaly, and the percent age of tear drop-like red blood cells in pre-CIMF were significantly lower than those in CIMF-Fs (P < 0.05). However, the number of hemoglobin and platelets in patients with pre-CIMF were significantly higher than that with CIMF-Fs (P < 0.01).</p><p><b>CONCLUSION</b>pre-CIMF and CIMF-Fs in clinical and histopathological features were different development stage of CIMF, while osteomyelosclerosis is a variant of CIMF, but not an independent disease.</p>

Natural killer-like T-cell lymphoma/leukemia--a case report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To identify the clinical and pathological features of natural killer-like T-cell lymphoma/leukemia.</p><p><b>METHODS</b>The characteristics of natural killer-like T-cell lymphoma/leukemia was discussed with report a new case and review of literatures.</p><p><b>RESULTS</b>A 16-year-old girl was referred to our hospital because of fever and disseminated cutaneous herpes and ulcer. Atypical lymphoid cells surrounded the dermal vessels with a CD3(+), CD8(+), CD4(-), CD5(-), CD10(-), CD19(-), CD57(-), CD56(+), perforin(+), granzyme B(+) immunophenotype and rearranged T-cell receptor-gamma gene implicated natural killer-like T cell origin. She was treated with prednisone and for several months. Then the patient developed progressive spleen enlargement with overt leukemia, which led to her eventual death.</p><p><b>CONCLUSIONS</b>Natural killer-like T-cell lymphoma/leukemia is a rare disease with distinctive clinical, histopathologic, and immuno phenotypic characteristics. Current treatment modalities are ineffective for most of the patients.</p>

The clinical features of hepatitis associated aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyse the proportion of hepatitis associated aplastic anemia (HAAA) in severe aplastic anemia (SAA) and its clinical features of HAAA.</p><p><b>METHODS</b>All newly diagnosed SAA cases in our department in the recent 5 years were analyzed. A case-control study was undertaken to investigate the differences of clinical and laboratory features between HAAA and non-hepatitis associated SAA (non-HASAA) patients.</p><p><b>RESULTS</b>The proportion of HAAA in SAA was 3.3%. There was no significant difference in PB cell counts, bone marrow hematopoiesis status and the amount of blood transfusion between HAAA and non-HASAA patients. Sera from 13 patients with HAAA were tested for antibodies to hepatitis viruses A, B, and C and hepatitis B surface antigen. Twelve (92.3%) of them had negative serologic results for the tests and only one (7.7%) had a positive result for HBsAg and HBeAg. Serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were decreased prior to the diagnosis in twelve (92.3%) of the 13 HAAA patients. The percentage of CD4(+) cells in HAAA patients was significantly lower than that in non-HASAA patients (P < 0.05). HAAA patients had higher percentages of CD8(+) cells (P < 0.05) and lower ratios of CD4(+)/CD8(+) (P < 0.05). The early infection rate of the HAAA patients was significantly higher than that of non-HASAA patients (84.6% vs 42.3%, P < 0.05), with different mortalities (61.5% vs 15.4%, P < 0.05). The 2-year survival rate of HAAA patients was significantly lower than that of non-HASAA patients (16.6% vs 83.2%, P < 0.01).</p><p><b>CONCLUSION</b>The proportion of HAAA in SAA was 3.3%. Most of HAAA were associated with non-A, non-B and non-C hepatitis virus. Compared with that of non-HASAA, the abnormality of T cell immunity of HAAA was more severe, with a higher frequency of early infection and a higher mortality rate.</p>

Clinical features and prognostic factors in primary myelofibrosis patients under 45 years old / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and hematological features and identify the prognostic factors associated with short-term survival in primary myelofibrosis (PMF) patients under 45 years old.</p><p><b>METHODS</b>The clinical manifestations, laboratory parameters, and survival were retrospectively analyzed in 56 PMF patients under 45 years old. Univariate analysis of prognostic factors was performed using Logrank test and multivariate analysis using COX model.</p><p><b>RESULTS</b>Of the 56 patients, 27 were males and 29 females. The range of age was from 20 to 45 years (median 38 years). 84% of the patients were anemic and 66% Hb < 100 g/L. 32% of the patients had constitutional symptoms including fever, night sweats and weight loss. The median survival was 69 months (95% CI 11-127). By univariate analysis, Hb < 100 g/L, platelet count < 100 x 10(9)/L, WBC < 10 x 10(9)/L, constitutional symptoms and the duration from first signs to diagnosis < or = 6 months were associated with poor prognosis. By multivariate analysis, only Hb < 100 g/L and constitutional symptoms were independently associated with short survival. With these two adverse prognostic factors, the patients could be separated into a high risk and a low risk groups, and the median survivals were 16 and 88 months, respectively (P < 0.001). Using these two factors to predict the less than 3-years survival for individual patient, the sensitivity, specificity and positive predictive value were 67%, 100% and 100%, respectively.</p><p><b>CONCLUSION</b>Hb < 100 g/L and constitutional symptoms in PMF patients under 45 years old were significantly associated with short survival and poor prognosis. These two prognostic factors enabled to separate patients into a high and a low risk groups. The survival of high-risk patients was less than three years.</p>

Morphologic and clinical study of 131 cases of plasma cell myeloma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the characteristics histologic and cytologic features and clinical usefulness of plasma cell myeloma (PCM) subtyping according to WHO PCM classification.</p><p><b>METHODS</b>Bone marrow biopsy plastic-embedded sections were stained with H-G-E and Gomori's stains, and bone marrow aspirate smears were stained with Wright's stain. The clinicopathologic findings were then analyzed.</p><p><b>RESULTS</b>Of the 131 cases with PCM, three types of growth patterns were noted: interstitial (21 cases, 16.0%), nodular (46 cases, 35.1%) and packed (64 cases, 48.9%). Besides, there were three cytologic subtypes: mature plasma cell type (43 cases, 32.8%), immature (81 cases, 61.8%) and pleomorphic (7 cases, 5.3%) types. The age of patients with mature plasma cell type was significantly higher than that of immature type (P = 0.005); and the number of tumour cells in bone marrow smears was significantly higher than that of immature type (P = 0.003). The numbers of WBC and platelets in peripheral blood were also significantly higher than that of pleomorphic type (P = 0.024, P = 0.002, respectively). On the other hand, the number of platelets in peripheral blood of immature type was significantly higher than that of pleomorphic type (P = 0.019). Marrow fibrosis was more frequently observed in immature type than in mature plasma cell type (P = 0.000). The incidence of marrow fibrosis and osteolytic lesions was higher in high risk group than in low risk group (P = 0.000, P = 0.023 respectively). Twenty-one cases (56.8%) of the 37 cases treated with MP or MP and M2 chemotherapeutic regimens showed good response. However, there was no significant difference in treatment response and survival between different subtypes.</p><p><b>CONCLUSIONS</b>Each subtype of PCM carries different clinicopathologic features in some aspects. The classification carries important value in pathologic diagnosis and probably in predicting prognosis.</p>

Clinical and laboratory study of a case of hematidrosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To study the clinical characteristics and pathogenesis of hematidrosis.</p><p><b>METHODS</b>Detailed clinical manifestations and natural history of a patient with hematidrosis were presented. A series of laboratory examinations were performed, and skin pathohistologic features and ultra microscopic structures were observed.</p><p><b>RESULTS</b>The episodes of skin bleeding occurred on any site of the body spontaneously and promptly. The skin surface bloody extravasation has identical cell components as that of peripheral blood. All the results of laboratory tests were normal except a positive Trousseau's test. Skin pathohistological study revealed some intradermal bleeding and emphraxised capillaries. No abnormality was found in sweat glands, hair follicles and sebaceous glands.</p><p><b>CONCLUSION</b>The pathological basis for hematidrosis might be a distinctive vasculitis.</p>

Observation on treatment of post-remission acute myeloid leukemia patients by lingxiong piaoling powder and longchan cigu decoction / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To explore the effect of the treatment for long-term disease-free survival (DFS) of post-remission patients with acute myeloid leukemia (AML).</p><p><b>METHODS</b>Twenty-nine AML patients with completely remission (CR) and 17 with partial remission (PR) were treated with Chinese medicine, Longchan Cigu Decoction 1 dose per day and Lingxiong Piaoling Powder 7 doses per month, and combined with DA or HA regimen of chemotherapy one course per year. Patients peripheral blood picture, bone marrow smear, biopsy and human leukocyte antigen DR (HLA-DR) cells were examined before and after treatment, and their disease-free survival (DFS) was followed up.</p><p><b>RESULTS</b>After treated with above mentioned treatment for 2 months, percentage of patients with normal peripheral blood count increased, including patients with WBC > or = 4.0 x 10(9)/L raised from 46% to 70%, with Hb > or = 120 g/L from 17% to 46% and with PLT > or = 100 x 10(9)/L from 63% to 85%; nucleated cell volume in bone marrow increased from 35.83 +/- 28.42% to 60.46 +/- 17.73% (P < 0.01); HLA-DR cell was also increased significantly from 10.55 +/- 4.95% to 14.84 +/- 4.94%, (P < 0.01); while the residual leukemia cells were not increased in one year, from 5.90 +/- 5.09% before and 5.82 +/- 2.42% after treatment (P > 0.05). The maximal DFS in patients was 123 months. The 3-year survival rate was 64.15 +/- 1.96% and 5-year survival rate was 51.19 +/- 16.25%.</p><p><b>CONCLUSION</b>The integrative Chinese and western medicine treating program used in this study is beneficial for the long-term treatment of AML patients after complete remission.</p>

Hemangioblastic characteristics of fetal bone marrow-derived Flk1+ CD34- cells / 中国医学科学院学报

<p><b>OBJECTIVE</b>To investigate whether fetal bone marrow stromal cells have hemangioblastic characteristics.</p><p><b>METHODS</b>Human fetal bone marrow stromal cells (hfMSCs) were isolated and cultured. Immunophenotypes of hfMSCs were tested by FACS. hfMSCs seeded in the matrigel were induced with vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in vitro. Vascularization and hematopoiesis were detected with immunohistochemistry and electron microscope.</p><p><b>RESULTS</b>The typical properties of this CD34- stromal cell population were that 99% cells expressed Flk1 (vascular endothelial cell growth factor receptor 2) and tube structure was formed. In the process of induction, hfMSCs could give rise to CD34+ round cells.</p><p><b>CONCLUSIONS</b>We have demonstrated that fetal bone marrow stroma-derived Flk1+ CD34- cells could differentiate into vascular endothelial cells and hematopoietic cells, indicating that fetal bone marrow stroma-derived Flk1+ CD34- cells have hemangioblastic characteristics.</p>