Hyperfibrinolysis in a patient with cellulitis.

A case of acute hyperfibrinolysis complication in cellulitis was reported. The bleeding did not stop for two days because of hematological defect. After giving antifibrinolysin and treating the etiologic factor, the bleeding stopped within a few hours. One should be aware that excessive bleeding in infection can possibly be caused by hyperfibrinolysis.     

Dyskeratosis congenita associated with hypocellular myelodysplastic syndrome: a case report

A 16-year-old female patient presented with complaints of malaise, dizziness, syncope, and nausea of 1-week duration. On dermatologic examination there were telangiectasias, atrophic areas, and poikiloderma with both hypopigmentation and hyperpigmentation on the neck and the proximal parts of the thighs. The bone marrow biopsy specimen showed hypocellularity and dysplastic megakaryocytic and erythroid elements, findings consistent with hypocellular myelodysplastic syndrome, which was further confirmed by cytogenetic studies. Thereafter, she was referred for allogeneic bone marrow transplantation.     

Inhibition of apoptosis by BCL2 prevents leukemic transformation of a murine myelodysplastic syndrome

Programmed cell death or apoptosis is a prominent feature of low-risk myelodysplastic syndromes (MDS), although the underlying mechanism remains controversial. High-risk MDS have less apoptosis associated with increased expression of the prosurvival BCL2-related proteins. To address the mechanism and pathogenic role of apoptosis and BCL2 expression in MDS, we used a mouse model resembling human MDS, in which the fusion protein NUP98-HOXD13 (NHD13) of the chromosomal translocation t(2;11)(q31;p15) is expressed in hematopoietic cells. Hematopoietic stem and progenitor cells from 3-month-old mice had increased rates of apoptosis associated with increased cell cycling and DNA damage. Gene expression profiling of these MDS progenitors revealed a specific reduction in Bcl2. Restoration of Bcl2 expression by a BCL2 transgene blocked apoptosis of the MDS progenitors, which corrected the macrocytic anemia. Blocking apoptosis also restored cell-cycle quiescence and reduced DNA damage in the MDS progenitors. We expected that preventing apoptosis would accelerate malignant transformation to acute myeloid leukemia (AML). However, contrary to expectations, preventing apoptosis of premalignant cells abrogated transformation to AML. In contrast to the current dogma that overcoming apoptosis is an important step toward cancer, this work demonstrates that gaining a survival advantage of premalignant cells may delay or prevent leukemic progression.     

A case of Legionella pneumonia with myelodysplastic syndrome]

A 40-year-old man was admitted with high fever and cough. Pneumonic shadows of the left middle and lower lung fields increased rapidly, and his blood gases worsened. Initial treatment with cefmenoxime, piperacillin, and minocycline was ineffective. Administration of rifampicin was started for suspected legionella pneumonia, but it did not control the spread of the pneumonia shadows. After addition of an antifungal agent and trimethoprim-sulfamethoxazole, his symptoms gradually improved. Isolation of Legionella pneumophila from sputum specimens collected on the 4th day of admission confirmed the diagnosis on day 10. The patient was then given oral rifampicin plus cefmenoxime to prevent mixed infection, and showed a satisfactory improvement. Legionella pneumonia developed secondary to compromise of the patient's immunity due to steroid therapy for MDS. After recovering from Legionella pneumonia, the patient subsequently developed tuberculous pleurisy and Pneumocystis carinii pneumonia, which were cured by antituberculous therapy and trimethoprim-sulfamethoxazole. However, acute hepatitis followed by hepatic failure developed, and he died on day 121 after admission.     

Mesenchymal stromal cells of myelodysplastic syndrome and acute myeloid leukemia patients have distinct genetic abnormalities compared with leukemic blasts

Mesenchymal stromal cells (MSCs) are an essential cell type of the hematopoietic microenvironment. Concerns have been raised about the possibility that MSCs undergo malignant transformation. Several studies, including one from our own group, have shown the presence of cytogenetic abnormalities in MSCs from leukemia patients. The aim of the present study was to compare genetic aberrations in hematopoietic cells (HCs) and MSCs of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) patients. Cytogenetic aberrations were detected in HCs from 25 of 51 AML patients (49%) and 16 of 43 MDS patients (37%). Mutations of the FLT3 and NPM1 genes were detected in leukemic blasts in 12 (23%) and 8 (16%) AML patients, respectively. Chromosomal aberrations in MSCs were detected in 15 of 94 MDS/AML patients (16%). No chromosomal abnormalities were identified in MSCs of 36 healthy subjects. We demonstrate herein that MSCs have distinct genetic abnormalities compared with leukemic blasts. We also analyzed the main characteristics of patients with MSCs carrying chromosomal aberrations. In view of these data, the genetic alterations in MSCs may constitute a particular mechanism of leukemogenesis.     

Immunophenotypic characterization of myelomonocytic cells in patients with myelodysplastic syndrome

Summary. Infections, an important determinating factor in the clinical course of myelodysplastic syndromes (MDS), result in activation of myelomonocytic cells. In this study we demonstrate activation-associated immunophenotypic changes of cell surface antigens on monocytes and granulocytes observed in two groups of MDS patients, one with low and another one with high clinical risk, and compared them to healthy individuals. Significantly changed expression of the complement receptors 1 (CD35) and 3 (CD11b), the Fcγ receptor I (CD64), the leucocyte-homing receptor (CD44) and the activation associated membrane proteins CD67 and M5 were found on monocytes and/or granulocytes of MDS patients. In low-risk MDS patients we observed activation-associated phenotypic changes only in monocytes, whereas in high-risk MDS patients, both monocytes and granulocytes showed such changes. Additionally, we performed respiratory burst experiments and observed an impaired response of monocytes and granulocytes derived from MDS patients. Despite the fact that all patients were free of infection by clinical criteria, cell surface phenotyping as well as the reduced respiratory burst capacity of myelomonocytic cells suggests in vivo preactivation of these cells.     

Therapy-related myelodysplastic syndrome in a case of cutaneous adult T-cell lymphoma

We report a case of therapy-related myelodysplastic syndrome (t-MDS) in adult T-cell lymphoma. A 69-year-old man suffered from cutaneous adult T-cell lymphoma, which was treated with radiation to the skin and combination chemotherapy of CHOP-V-MMV and VEPA-B. After 14 months of these therapies, anemia and thrombocytopenia appeared, and bone marrow aspiration smears showed immature myeloblasts, dysplastic erythroblasts, and micromegakaryocytes. Therapy-related MDS of refractory anemia with an excess of blasts was diagnosed. Cytogenetic study of the bone marrow cells showed 5q- and additional abnormalities. Rearrangement of the MLL gene was observed in the bone marrow cells. Mutations of N-ras codons at 12,13, and 61, p53 tumor suppressor gene, and monoclonal integration of human T-lymphotrophic virus -1 provirus DNA were not observed in the bone marrow cells. The patient died of pneumonia 21 months after diagnosis of cutaneous adult T-cell lymphoma.     

血行播散性结核病并发骨髓增生异常综合征一例——文献复习及临床诊治过程分析

血行播散性结核病是一种重症结核病,临床症状常不典型,影像学诊断报告也常常滞后,早期诊断困难。骨髓增生异常综合征(MDS)是一种恶性血液病,早期临床症状和血液系统异常表现一般不具有特异性。血行播散性结核病并发MDS常导致病情互相掩盖,易漏诊、误诊。笔者报道2017年4月首都医科大学附属北京胸科医院以“头晕、乏力3个月,发热2个月” 收治入院的1例患者,初步诊断为血行播散性结核病并发全血细胞严重减少,早期经抗结核药物治疗后临床症状、肺部病变及血液系统表现均一度好转,但后期在肺部病变进一步好转的同时,血液系统异常却再次加重,最终经多次骨髓穿刺检查诊断为血行播散性结核病并发MDS。笔者希望通过对诊治过程的分析讨论,提高临床医生对两病并存的诊断认识,分享治疗经验。     

A case of myelodysplastic syndrome associated with IgA nephropathy]

A 72-year-old man was admitted for examination of dyspnea and pitting edema of the lower legs in July, 1996. His hemoglobin level was 6.9 g/dl, and myelodysplastic syndrome (MDS) was revealed by bone marrow aspiration, and frequent transfusions were needed. His renal function rapidly deteriorated in the middle of August (BUN 45 mg/dl, Cr 4.8 mg/dl) and IgA nephropathy (IgAN) with marked intestinal nephritis was disclosed by renal biopsy. In November, joint manifestations of warmth and pain, which suggested arthritis, appeared at the bilateral wrist and ankle joints. Soon after receiving prednisolone (20 mg/day), the arthritis was relieved. Renal function also improved (BUN 41 mg/dl, Cr 21 mg/dl) and frequent transfusions were no longer necessary. This is a case with various clinical manifestations of MDS, IgAN, and arthritis, and appears to be the first MDS case complicated with IgAN. A number of case reports have identified immune abnormalities in patients with MDS. Immune and bone marrow abnormalities have been reported to be involved in the pathogenesis of IgAN. Thus, MDS could be complicated by IgAN. Their pathogenetic association is discussed in this paper.     

A case of myelodysplastic syndrome complicated with organizing pneumonia]

A 74-year-old man had been given a diagnosis of myelodysplastic syndrome (MDS), and had been treated with granulocyte-colony stimulating factor (G-CSF). 1 year later, he suffered from fever and his chest X-ray lung biopsy did not provide a diagnosis, video-assisted thoracoscopic lung biopsy was performed, which yielded a histological diagnosis of organizing pneumonia. His pulmonary disease was diagnosed as secondary organizing pneumonia due to MDS, and was treated successfully with steroids. Vigorous efforts to establish a histological diagnosis is needed for the antibiotics-resistant pneumonia in the case of MDS.     

A case of pulmonary manifestation associated with myelodysplastic syndrome]

A 69-year-old woman with myelodysplastic syndrome (MDS) was admitted to our hospital because of recurrent fever and pulmonary infiltration shadows. On the seventh day of hospitalization, she had an attack of high fever and cough and laboratory tests revealed an elevated leukocyte count and elevated serum C-reactive protein. Chest radiographs showed infiltration shadows in the right middle and lower lung fields. Because a diagnosis of bacterial pneumonia was initially suggested, she was treated with antibiotics. However, the infiltration shadows on the chest radiograph had not improve, so bronchofiberscopy was performed. Analysis of fluid obtained by bronchoalveolar lavage (BAL) showed an increase in the total cell count, predominantly in lymphocytes and neutrophils. A transbronchial biopsy specimen showed infiltration of numerous neutrophils with necrosis under the bronchial epithelium, and edematous septa were infiltrated with numerous neutrophils and lymphocytes. BAL, blood, urine, bone marrow, and sputum cultures were all free of bacteria, mycobacteria and fungi. Interstitial infiltration by numbers of neutrophils associated with MDS was diagnosed and steroid treatment was performed.