C/EBPα in multiple myeloma patients may lead to increased hepcidin / 中国实验血液学杂志

This study was aimed to explore the possible mechanisms of hepcidin increase in multiple myeloma patients. The clinical information and peripheral venous blood of eligible patients with previously untreated multiple myeloma were collected. Serum concentration of IL-6 was detected by ELISA. Peripheral blood monocytes were isolated by CD14⁺ magnetic beads. The expression of hepcidin, IL-6 and C/EBPα mRNA of monocytes were detected by real time quantitative PCR. The results indicated that the hemoglobin level was reduced in 17 multiple myeloma patients enrolled in study (97.8 ± 27.5 g/L), showing the characteristics of anemia of chronic disease. The hepcidin and C/EBPα expression of peripheral blood monocytes significantly increased (P < 0.01), serum IL-6 was also higher than that in normal controls (P < 0.01). Serum IL-6 positively correlated with monocyte hepcidin and C/EBPα expression (P < 0.05); monocyte C/EBPα expression positively correlated with monocyte hepcidin expression (P < 0.05). It is concluded that the elevated IL-6 may induce hepcidin expression through up-regulating C/EBPα in untreated myeloma patients.

Prevalence and features of pathogenic bacteria in the department of hematology without bone marrow transplantation in Peking Union Medical College Hospital from 2010 to 2012 / 中国医学科学院学报

<p><b>OBJECTIVE</b>To investigate the incidence, pathogens, and clinical features of infection in consecutive cases from 2010 to 2012 in Peking Union Medical College Hospital.</p><p><b>METHOD</b>The incidence, pathogen, treatment, and outcomes of patients with hematological diseases who had positive findings of bacterium in their samples from 2010 to 2012 were retrospectively analyzed.</p><p><b>RESULTS</b>There were 449 positive samples (5.8%) from 4 890 patients during this period, among which 388 were proved to be with pathogenic bacteria. Samples separated from patients with community-aquired infections accounted for 8.4% of all positive samples. Most community-aquired infections were caused by Gram-negative bacteria (75%), although no multidrug-resistant bacteria was observed. Samples separated from patients with nosocomial infections accounted for 91.6% of all positive samples. Respiratory tract (49.4%) and peripheral blood (32.6%) were the most common samples with positive results. Skin soft tissues (10.4%), and urine (3.7%) were less common samples. Most of the pathogenic bacteria of the nosocomial infections were Gram-negative (66.9%). The most common Gram-negative bacteria included Escherichia coli (13.8%), Pseudomonas aeruginosa (12.1%), and Klebsiella pneumonia (12.1%), while Staphylococcus aureus (10.4%), Enterococcus faecium (7.0%), and Staphylococcus epidermidis (5.1%) were the most common Gram-positive bacteria. Gram-negative bacteria consisted of most of sputum samples and peripheral blood samples. Samples from the surface of skin wound and anal swab were composed largely by Gram-positive bacteria (63.8%). The detection rates of extended-spectrum beta-lactamase-producing Klebsiella pneumonia/Klebsiella oxytoca, Escherichia coli, and Proteus mirabilis were 24.0%, 87.9% and 38.4%, respectively. The resistance to Acinetobacter baumannii was serious. Multidrug-resistant, extensive drug resistant and pan drug resistant A. baumannii acountted for 74% of all A. Baumannii infections. Stenotrophomonas maltophilia showed low resistance to sulfamethoxazole/trimethoprim, levofloxacin and minocycline. Also, 22 methicillin-resistant Staphylococcus aureus and 9 methicillin-resistant Staphylococcus Epidermidis were detected, which were only sensitive to vancomycin, teicoplanin, and linezolid. All patients were treated in the haematology wards and most of them were under agranulocytosis or immunosuppression. Finally, 22 patients reached clinical recovery through anti-infective therapy, whereas 49 patients died. Among those deaths, 42 patients attributed to severe infections and infection-associated complications. Fourteen of all the deaths might be infected with drug-resistance bacteria. There were 61 samples proved to be bacteria colonization. Nonfermenters such as Acinetobacter baumannii and Stenotrophomonas maltophilia made up for a large amount of bacteria colonization.</p><p><b>CONCLUSIONS</b>The pathogens of nosocomial infections in the hematology ward are mainly Gram-negative bacteria. The incidences and pathogens vary from different infection sites. Nosocomial infection still has a higher mortality rate. Once nonfermenters are detected positive, the pathogenic or colonial bacteria should be distinguished.</p>

Peripheral blood monocyte hepcidin in patients with multiple myeloma is associated with anemia of chronic disease / 中国实验血液学杂志

Disorders of iron utilization caused by abnormal elevation of hepcidin levels are the main mechanism of anemia of chronic disease. Hepcidin is mainly produced by the liver. Recently it has been found that monocytes are another source of hepcidin. The increased hepcidin in serum and urine of multiple myeloma patients may be one cause of anemia of chronic disease (ACD). However it is unclear whether the peripheral blood monocyte hepcidin is involved in the pathogenesis of anemia of chronic disease. This study was purposed to investigate the role of monocyte hepcidin in multiple myeloma patients with anemia of chronic disease. The clinical data and peripheral venous blood of multiple myeloma patients were collected.Serum concentration of IL-6 and TNF-α was detected by ELISA. Peripheral blood monocytes were isolated by CD14(+) magnetic beads. Hepcidin, IL-6 and TNF-α mRNA of monocytes were detected by real time quantitative PCR. The results showed that the expression level of monocyte hepcidin mRNA in myeloma patients was higher than that in normal controls. In untreated patients, the expression level of monocyte hepcidin mRNA was negatively correlated with hemoglobin, and positively correlated with serum ferritin and IL-6 levels, but unrelated with TNF-α levels.It is concluded that the increased monocyte hepcidin levels in multiple myeloma patients may play an etiologic role in ACD.

Efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To analyze efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis (LCH).</p><p><b>METHODS</b>Clinical features and efficacy of radiotherapy for biopsy-proven adult patient with LCH from January 2000 to October 2012 in our hospital were retrospectively analyzed.</p><p><b>RESULTS</b>Seventeen (11 male and 6 female) adult LCH patients with a mean age of 31 (18-56) years old were treated by irradiation, all patients presented as single-system disease. The mean duration from diagnosis to irradiation was 8.3 (0-108) months. Although 12 of 17 patients (70.6%) had short-term response to radiotherapy, all patients but one (94.1%) progressed during long-term follow-up, the mean progression-free survival (PFS) was 14 (0-131) months. Of the progressed patients, one relapsed in situ, the remaining 15 patients progressed outside the irradiated region. Thirteen patients (76.5%) eventually progressed to multisystem disease.</p><p><b>CONCLUSION</b>Though radiotherapy for LCH in adults produced a high short-term response up to 70.6%, most of patients eventually progressed in situ or outside the irradiation region during long-term follow-up.</p>

Effect of erythropoietin on proinflammatory factors of human monocytes and its mechanisms / 中国实验血液学杂志

Erythropoietin (EPO) is the major means of treating anemia of chronic disease (ACD) through stimulating hematopoiesis, inhibiting hepcidin and decreasing proinflammatory factors. Recently, it has been found that monocytes are another source of hepcidin. EPO can reduce the hepcidin stimulated by IL-6 in monocytes, it is assumed that EPO can reduce hepcidin indirectly by reducing IL-6. However, the specific mechanism of EPO inhibiting the proinflammatory cytokines in monocytes is unclear now. This study was purposed to investigate the effect of EPO on monocyte proinflammatory factors and its molecular mechanism. IL-6 mRNA and TNF-α mRNA were detected by real time PCR, level of signaling molecule PARP-1 protein was detected by Western blot. THP-1 monocytes were stimulated by 1 µg/ml lipopolysaccharide (LPS) to observe the impact of EPO at different concentrations (0.5, 1, 2, 5, 10 U/ml) for different time (0, 3, 6, 12, 24 hours) on the expression of IL-6 mRNA, TNF-α mRNA and PARP-1 protein. 1 µg/ml or 5 µg/ml EPO receptor (EPOR) antibody and/or 3-aminobenzamide (3-AB, PARP-1 inhibitor) were added to observe the antagonistic effect on EPO and the impact on PARP-1. The results showed that LPS could stimulate the THP-1 cells. EPO could decrease the levels of IL-6 and TNF-α stimulated by LPS in a dose- and time-dependent manners. The most significant decrease in IL-6 mRNA expression was observed in 2 U/ml EPO for 6 hours. And down-regulation of TNF-α mRNA expression was pronounced at 10 U/ml EPO for 3 hours. IL-6 mRNA expression could be stimulated by LPS, PARP-1 protein was induced at the same time. EPO inhibited the expression of IL-6 mRNA, while PARP-1 protein also decreased. Down-regulation of IL-6 mRNA and PARP-1 protein level was pronounced at 2 U/ml EPO for 6 hours. 3AB is a direct inhibitor of PARP-1. Similar to 3AB, EPO receptor antibody could antagonize the decline of IL-6 induced by EPO. It is concluded that EPO can inhibit the expression of IL-6 and TNF-α in monocytes, and the inhibition of IL-6 expression may be associated with decrease of PARP level.

Porcine anti-human lymphocyte globulin plus cyclosporine A therapy for severe aplastic anemia / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy of porcine anti-human lymphocyte globulin (P-ALG) plus cyclosporine A (CsA) therapy for severe aplastic anemia (SAA).</p><p><b>METHODS</b>Forty-eight SAA patients (31 males, 17 females) including 17 very severe aplastic anemias (vSAA) were treated with ALG plus CsA between 1999 to 2009 in our hospital and the outcomes were analyzed retrospectively for early mortality, response rate and quality, survival rate, toxicity and complications.</p><p><b>RESULTS</b>The median age was 28 (13 - 64) years. The interval from diagnosis to treatment was 45 days. The median neutrophil count at diagnosis was 0.178 × 10(9)/L. Overall response was 83.3% (54.2% complete, 29.2% partial) with a median time of 90 (23 - 380) days. 10.4% died of infection within 30 days mainly of fungi infection. Only 1 patient relapsed 2 years after treatment. No clonal disease was found. The 1.5-year survival rate was 87.5%. vSAAs had less response, higher early mortality and less survival (64.7%, 29.4% and 51.8%, respectively) compared to that of SAA (93.5%, 0, 100%, respectively, P < 0.05). Grouped patients with different age, gender, intervals between diagnosis and treatment and pre-existing infections had similar response. The main side effects were fever and skin rash (52.1%), serum sickness (16.7%), impaired liver function (60.4%) and hemorrhage (2.1%). No treatment-related mortality was found.</p><p><b>CONCLUSION</b>P-ALG plus CsA is an ideal and well tolerated treatment for SAA but not for vSAA.</p>

The effects and mechanisms of erythropoietin on hepcidin of human monocytes / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the in vitro effect of erythropoietin (EPO) on hepcidin of monocytes and its molecular mechanisms.</p><p><b>METHODS</b>Hepcidin and signaling molecules including C/EBPalpha, Smad1/5/8, p-Smad1/5/8 and p-STAT3 were detected by real time PCR and Western blot. THP-1 monocytes were stimulated by interleukin-6 (IL-6) or lipopolysaccharide (LPS). EPO receptor (EPOR) antibody was added to observe its antagonistic effect on EPO and impact on the signaling proteins.</p><p><b>RESULTS</b>EPO suppressed mRNA expression of THP-1 hepcidin of monocytes induced by 20 ng/ml IL-6 or 1 microg/ml LPS in both dose and time dependent manner. The most decrease of hepcidin expression was observed at 2 IU/ml EPO for 6 hours. EPO also down-regulated hepcidin protein induced by 20 ng/ml IL-6. At 2 IU/ml EPO for 6 hours hepcidin protein was down-regulated, as was C/EBPalpha, p-Smad1/5/8 and p-STAT3. Antibody to EPOR antagonized the down-regulation of EPO on hepcidin and signaling proteins.</p><p><b>CONCLUSIONS</b>Monocytes hepcidin can be reduced by EPO when stimulated by IL-6 or LPS. The mechanism of which may be at least in part, via suppression of C/EBPalpha, p-Smad1/5/8 and p-STAT3 signaling.</p>

Effect of recombinant human erythropoietin on hepcidin mRNA expression in patients with multiple myeloma / 中国实验血液学杂志

This study was purposed to investigate the effect of multiple myeloma patients' sera on hepcidin mRNA expression of Hep-3b hepatoma cell line and effect of human interleukin-6 (IL-6) antibody or recombinant human erythropoietin (rhEPO) on hepcidin mRNA expression. The clinical information and serum of multiple myeloma patients were collected. Their sera of a final concentration of 10% were added into Hep-3b cell medium. The mRNA from Hep-3b cells was extracted, and hepcidin mRNA expression was detected by semi-quantitative reverse transcription polymerase chain reaction (RT-PCR). A final concentration of 10 ng/ml human IL-6 antibody and 2 U/ml rhEPO were added into the medium respectively. The results showed that the sera of untreated multiple myeloma patients elevated hepcidin mRNA expression of Hep-3b cells, compared with healthy controls and iron deficiency anemia patients. This effect was fully neutralized by human IL-6 antibody or rhEPO. The hemoglobin (Hb) level was stable during the follow up of regularly treated multiple myeloma patients and the effect of MM patient serum on Hep-3b cell hepcidin mRNA expression was reduced. It is concluded that the hepcidin mRNA expression of Hep-3b cell can be increased by untreated multiple myeloma patient serum. This promotive effect can be antagonised by IL-6, which suggests that IL-6 may be possible to elevate expression level of hepcidin in Hep-3b cells and results in anemia of chronic disease (ACD). The above mentioned promotive effects also can be suppressed by rhEPO, which indicates that the rhEPO may possess curative effect for ACD disease. During short-term follow-up of treated patients with multiple myeloma the Hb level is stable, the influence of patients serum on hepcidin mRNA of Hep-3b cells decreases, which shows the stabilization of disease and amelioration of ACD patient status.

Preliminary study of autologous peripheral blood stem cell transplantation in patients with POEMS syndrome / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of autologous peripheral blood stem cell transplantation (auto-PBSCT) after high dose melphalan in patients with POEMS syndrome.</p><p><b>METHODS</b>Nine patients including 6 males and 3 females received 10 auto-PBSCT after high dose melphalan in our hospital from June 2005 to October 2009. The median age at transplantation was 44 (39 - 48) years. The median time from onset of disease to transplantation was 12 (5 - 60) months. Peripheral stem cells were mobilized by G-CSF alone in one patient and 8 patients by G-CSF plus chemotherapy. Two patients were conditioned by melphalan 140 mg/m(2) and 7 by melphalan 200 mg/m(2). The median number of MNC was 3.75 (1.05 - 8.33) × 10(8)/kg, and that of CD34(+) cell was 5.37 (1.32 - 10.90) × 10(6)/kg.</p><p><b>RESULT</b>One patient received tandem auto-PBSCT and others received single one. Stem cell engrafted in all but 1 patient who died of severe infection and acute renal failure on day 9 after transplantation. Eight patients were evaluable for response. The median time to ANC ≥ 0.5 × 10(9)/L and platelet ≥ 20 × 10(9)/L was 10 (9 - 11) and 11.5 (9 - 14) days respectively. Two patient reached negative immunofixation electrophoresis (IFE) after stem cell mobilization and transplantation respectively, and the other 6 remained IFE postive after auto-PBSCT. Skin changes and edema of lower extremities were improved in 5 of 6 patients, lymphadenopathy relieved in 1 and papilledema improved in 2 of 3 patients. All but 1 patient achieved gradual neurologic improvement after transplantation.</p><p><b>CONCLUSION</b>PBSCT is an effective and safe therapy for POEMS syndrome patients with low treatment related mortality.</p>

Fcgamma receptor IIIA polymorphisms and efficacy of rituximab therapy on Chinese diffuse large B-cell lymphoma / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Rituximab is used extensively in combination with chemotherapy to cure non-Hodgkin's lymphoma (NHL), and not only accelerates short-term improvement, but also prolongs patient survival and decreases receptor relapse. The aim of this study was to evaluate the impact of Fcgamma IIIA (FcgammaRIIIA) gene polymorphisms on the response to rituximab therapy for newly diagnosed B-cell lymphomas.</p><p><b>METHODS</b>Patients with newly diagnosed histologically-proven CD20-positive B-cell lymphoma were eligible for the study. All of the patients received rituximab combined with chemotherapy (CHOP). The FcgammaRIIIA type was analyzed by PCR. The initial efficacy was assessed after 6 cycles and the long-term survival was determined.</p><p><b>RESULTS</b>Thirty-four patients were recruited between October 2005 and April 2006. The FcgammaRIIIA distribution was as follows: 11 patients were VV, 5 were FF, and 18 were VF. After a median of 6 cycles (range 4-8) of rituximab combined chemotherapy, the overall response rate was 79% (82% in the VV group, 83% in the VF group, and 60% in the FF group; P=0.04). After a median follow-up time of 37 months (range 34-41), there were 12 relapses among 27 responders (44%); 5 of 9 patients (5/9) in the VV group, 5 of 15 patients (33%) in the VF group, and 2 of 3 patients (2/3) in the FF group (P=0.21). The 1-year overall survival in the VV, FF, and VF groups was 80%, 60%, and 80%, respectively, and the 3-year overall survival was 58%, 40%, and 69%, respectively (P=0.08). After analysis by COX regression, only the international prognosis index and response to initial treatment were significantly related to overall survival.</p><p><b>CONCLUSIONS</b>The distribution of FcgammaRIIIA polymorphisms in this B-cell lymphoma population shows that VF is most frequently expressed, followed by VV and FF. Patients with the FcgammaRIIIA VV and VF types are more sensitive to the initial treatment of rituximab combined with chemotherapy and have superior long-term survival compared with those with FF. Nevertheless, FcgammaRIIIA polymorphisms do not predict prognosis independently.</p>

Comparison of efficacy and adverse effects between arsenic trioxide and all-trans retinoic acid in patients with acute promyelocytic leukemia / 中国医学科学院学报

<p><b>OBJECTIVE</b>To compare the efficacy and adverse effects between arsenic trioxide (ATO) and all-trans retinoic acid (ATRA) in patients with acute promyelocytic leukemia (APL).</p><p><b>METHODS</b>The clinical data of 71 patients with newly diagnosed APL were retrospectively analyzed. Two groups were classified according to the induction regimens, namely ATO group (n = 41) and ATRA group (n = 30). The complete remission (CR) rate and the time to CR were compared between these two groups.</p><p><b>RESULTS</b>The CR rate was 97.5% in ATO group and 93.3% in ATRA group (P > 0.05). The median time to CR was 29 days (21-45 days) in ATO group, which was significantly shorter than 38.5 days (24-63 days) in ATRA group (P < 0.001). Retinoic acid syndrome occurred in 52.9% of patients treated with ATRA, which affected the further use of ATRA.</p><p><b>CONCLUSIONS</b>Both ATO and ATRA have high response rates for newly diagnosed patients with APL. Compared with ATRA, ATO induction therapy has shorter time to achieve CR and less adverse effects, and therefore may be the first-line therapy for APL.</p>

Blood concentration monitoring during high-dose methotrexate treatment / 中国医学科学院学报

<p><b>OBJECTIVE</b>To explore the clinical value of blood concentration monitoring during high-dose methotrexate (MTX) treatment.</p><p><b>METHODS</b>High-dose MTX (1.5-9.0 g) was infused to 105 patients with acute lymphoblastic leukemia or lymphoma, and then the blood MTX concentration was measured by fluorescence polarization immune assay (FPIA) 44 hours after the start of administration. The procedure was repeated every 6-12 hours until the concentration was less than 0.1 micromol/L.</p><p><b>RESULTS</b>Forty-four hours after the start of administration, the blood MTX concentration (C(MTX/44h)) was > or = 5 micromol/L in 6 patients (2.8%) and was between 1 and 5 micromol/L in 23 patients (10.6%). C(MTX/44h) > or = 1 micromol/L was more common in patients received 5.0 g MTX. No severe adverse event was observed in all patients.</p><p><b>CONCLUSIONS</b>Blood MTX concentration is different after high-dose MTX treatment due to individual metabolic differences, and therefore it is clinically important to monitor blood concentration of MTX. Elimination delay is more common in patients receive 5.0 g MTX. Application of high-dose MTX therapy under the monitoring of blood MTX concentration is safe and feasible.</p>

Efficacy and safety of high-dose dexamethasone-based regimens in the newly diagnosed multiple myeloma patients with renal impairment / 中国医学科学院学报

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of high-dose dexamethasone-based regiments in newly diagnosed multiple myeloma patients with renal impairment.</p><p><b>METHODS</b>The clinical data of 22 patients with newly diagnosed multiple myeloma patients with renal impairment who received high-dose dexamethasone-based regiments from August 2006 to August 2008 in Peking Union Medical College Hospital were retrospectively reviewed.</p><p><b>RESULTS</b>After receiving a median 4 cycles of high-dose dexamethasone-based regiments, renal impairment was reversed in 7 patients (31.8%) with a median time to reversal of 31 days. Sixteen patients (72.7%) achieved overall response, including 7 patients (31.8%) had complete remission / near complete remission. The grade 3 or 4 adverse events included neutropenia (13.6%), infections (22.7%), peripheral neuropathy (9.1%), and ileus (4.5%).</p><p><b>CONCLUSION</b>The high-dose dexamethasone-based regiments are safe and effective for newly diagnosed multiple myeloma patients with renal impairment.</p>

Clinical features of invasive pulmonary fungal infection secondary to malignant blood diseases / 中国医学科学院学报

<p><b>OBJECTIVE</b>To summarize the clinical features of invasive pulmonary fungal infection (IPFI) secondary to malignant blood diseases (MBD).</p><p><b>METHODS</b>We retrospectively analyzed the clinical data of 52 patients with IPFI secondary to MBD admitted to Peking Union Medical College Hospital from January 1995 to December 2008.</p><p><b>RESULTS</b>The incidences of IPFI secondary to acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), non-Hodgkin's lymphoma (NHL), and aplastic anemia (AA) were 4.6%, 3.2%, 2.8%, and 2.5%, respectively. In patients with IPFI secondary to AML, 88.5% (23/26) of the patients suffered from the infections during the non-remission (NR) period (including relapse), and 11.5% (3/26) in the complete-remission (CR) period. In all the patients with IPFI secondary to malignant blood diseases, 86.5% (45/52) of MBD were neutropenic or agranulocytic, and 67.3% (35/52) had been treated with broad-spectrum antibiotics for more than 96 hours before anti-fungal therapy. The total mortality after anti-fungal therapy was 13.7% (7/51). More than half of patients with fluconazole or itraconazole as the first-line therapy had to switch to other medicines because of poor infection control.</p><p><b>CONCLUSIONS</b>IPFI secondary to MBD is most common in AML patients. Patients with NR of AML, neutropenia or agranulocytosis, and long-term broad-spectrum antibiotics usage are susceptible to IPFI. Fluconazole and itraconazole have low efficacy, and other more potent anti-fungal medicines should be considered.</p>

Dendritic cell sarcoma: 4 cases report with literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To describe the clinical and pathological features, treatment and prognosis of dendritic cell sarcoma (DCS).</p><p><b>METHODS</b>A group of DCS was described, including two cases of follicular dendritic cell sarcoma (FDCS), one each of interdigitating dendritic cell sarcoma (IDCS) and langerhans cell sarcoma (LCS). The related English literatures were reviewed.</p><p><b>RESULTS</b>Two patients with IDCS were a 19-year-old man and a 45-year-old woman respectively, both exhibited fever of unknown origin and painless lymphadenopathy. Pathological diagnosis of lymph node biopsy was FDCS with positive CD21 and CD35. Both patients achieved complete remission (CR) after 6 cycles of chemotherapy (CHOP: cyclophosphamide, epirubicin, vindesine, and prednisolone). However, the male patient relapsed 5 months later and another patient was still in CR at 5 months follow-up. One case of IDCS was a 42-year-old man, who manifested as paraneoplastic pemphigus. Biopsy of mediastinal lymph node demonstrated IDCS and immunohistochemistry showed positive S-100 staining. This patient died of pneumonia after two cycles of CHOP. One patient of LCS was a 54-years-old woman with fever, painless lymphadenopathy and diffused pulmonary nodules. The diagnosis of LCS was established after excisional biopsy was taken from inguinal lymph node. Positive staining of CD1a and S-100 was displayed by immunohistochemistry. Electron microscope examination confirmed the presence of Birbeck granule in tumor cells. Four cycles of chemotherapy (including ECHOP, FND) were administered, but the disease progressed.</p><p><b>CONCLUSION</b>DCS is a group of very rare sarcoma, FDCS, IDCS and LCS have different characteristic clinical features, immunophenotype and prognosis. The prognosis of most patients is poor.</p>