Trichosporonosis is a potentially life-threatening infection with Trichosporon beigelii, the causative agent of white piedra. The systemic infection by this fungus has been most frequently described in immunocompromised hosts with neutropenia. Here, we report the first patient with disseminated infection by T. beigelii in Korea, acquired during a period of severe neutropenia after chemo-therapy for myelodysplastic syndrome. The patient recovered from the infection after an early-intensified treatment with amphotericin B and a rapid neutrophil recovery. The disseminated infection by T. beigelii is still rare, however, is an emerging fatal mycosis in immunocompromised patients with severe neutropenia.
Adult ; Amphotericin B/therapeutic use ; Human ; Male ; Mycoses/drug therapy/*etiology ; Myelodysplastic Syndromes/*complications/drug therapy

No abstract available.
Adult ; Behcet Syndrome/*complications/therapy ; Colitis/*etiology/therapy ; Female ; *Hematopoietic Stem Cell Transplantation ; Humans ; Myelodysplastic Syndromes/*complications/therapy ; Transplantation, Homologous

Female ; Humans ; Leukemia, Myeloid, Acute ; complications ; etiology ; Middle Aged ; Myelodysplastic Syndromes ; complications ; etiology ; Uterine Cervical Neoplasms ; complications ; drug therapy ; radiotherapy

Shwachman-Diamond syndrome (SDS) is a rare genetic disorder of unknown pathogenesis involving exocrine pancreatic insufficiency and hematological and skeletal abnormalities. About 25% of patients develop hematopoietic malignancies. We report on a case of acute myeloid leukemia (M2) in a 21-year-old woman affected by SDS. She was treated with conventional chemotherapy (idarubicin plus cytarabine) and reached complete remission of leukemia. After induction chemotherapy, she underwent allogeneic bone marrow transplantation (BMT). The BMT preparative regimen consisted of total body irradation (TBI) followed by cyclophosphamide. Cyclosporin A and short term methotrexate were used for graft-versus-host disease prophylaxis. After a follow-up of 12 months, she is alive leukemia free off any immunosuppressive agent. Although experience in this field is scarce, we speculate that bone marrow failure in SDS is an indication for BMT which is the only curative trentment option.
Adult ; *Bone Marrow Transplantation ; Case Report ; *Cell Transformation, Neoplastic ; Female ; Human ; Leukemia, Myelocytic, Acute/*pathology/*therapy ; Myelodysplastic Syndromes/*complications/*therapy ; Pancreatic Insufficiency/complications/therapy ; Syndrome ; Transplantation, Homologous

Anemia, Refractory, with Excess of Blasts ; complications ; drug therapy ; Azacitidine ; analogs & derivatives ; therapeutic use ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes ; complications ; drug therapy ; Treatment Outcome

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Azacitidine ; administration & dosage ; analogs & derivatives ; Female ; Humans ; Leukemia, Myeloid, Acute ; drug therapy ; etiology ; Male ; Myelodysplastic Syndromes ; complications ; pathology ; Treatment Outcome

Myelodysplastic syndrome (MDS) is a heterogenous group of stem cell disorders usually characterized by progressive refractory cytopenias, which could progress to acute myeloid leukemia. MDS may be associated with a wide spectrum of skin lesions, including neoplastic cell infiltration, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, vasculitis, and panniculitis. However, erythema nodosum is rarely associated with MDS. Unusual rheumatologic manifestations in patients with MDS also have been reported, which range from asymptomatic serological abnormalities to classic connective tissue disorders such as Sjogren's syndrome, relapsing polychondritis, systemic lupus erythematosus, rheumatoid arthritis and mixed connective tissue disease. However, concurrent erythema nodosum and serositis has rarely been reported. We describe a case of MDS with erythema nodosum and immune-mediated pericardial effusion in a 34-year-old woman.
Adult ; Biopsy ; Diagnosis, Differential ; Erythema Nodosum/*complications/diagnosis/drug therapy ; Female ; Follow-Up Studies ; Glucocorticoids/therapeutic use ; Humans ; Myelodysplastic Syndromes/*complications/diagnosis/drug therapy ; Prednisone/therapeutic use ; Serositis/*complications/diagnosis/drug therapy ; Tomography, X-Ray Computed

OBJECTIVETo study the clinical effect of combined treatment with gamma-globulin, corticosteroids, immunosuppressor and Chinese medicines on systemic lupus erythematosus (SLE) patients characterized by hypoplastic bone marrow (HBM).

METHODSNineteen patients were randomly assigned to two groups, the treated group (10 cases) received combined therapy of prednisone and cyclophosphamide (CTX) plus Chinese medicine Langchuang Recipe after being treated impactively with gamma-globulin. The control group (9 cases) was treated with prednisone and CTX. Changes of hypoplastic bone marrow, peripheral white blood cell (WBC), complement C3, 24 h urinary protein excretion, and lupus activity index (LAI) were observed, and a follow-up was carried out for one year.

RESULTSAfter one-month treatment, the bone marrow hypoplasia was relieved significantly in the treated group (P < 0.05, P < 0.01), showing an improvement superior to that in the control group (P < 0.05, P < 0.01); after three months treatment, the level of complement C3 increased (P < 0.01), while the 24 h urinary protein excretion and LAI decreased in the treated group (P < 0.05), showing significant difference as compared with those in the control group (P < 0.05, P < 0.01). In the follow-up period, 3 cases withdrew from the trial because of infection and 4 cases manifested full moon-face and acne in the control group, while no adverse reaction was found in the treated group.

CONCLUSIONTreatment with integrated Chinese and Western medicine could effectively improve bone marrow hypoplasia, alleviate the clinical symptoms, suppress the activity of lupus in patients, and reduce the adverse reaction of treatment, showing a superiority to the treatment with prednisone combining with CTX.

Adult ; Cyclophosphamide ; therapeutic use ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; drug therapy ; Male ; Myelodysplastic Syndromes ; complications ; drug therapy ; Phytotherapy ; Prednisone ; therapeutic use ; Treatment Outcome ; gamma-Globins ; therapeutic use

A 32-yr-old male diagnosed with myelodysplastic syndrome underwent an unmanipulated, unrelated, HLA matched, peripheral blood stem cell transplantation. The patient and donor were both blood type O, CcDEe. Twelve weeks post-transplantation, he developed acute autoimmune hemolytic anemia (AIHA). He was transfused multiple times with washed O red cells. High-dose steroid therapy was initiated and he underwent splenectomy; however, AIHA was refractory to therapy. The patient was further treated with combined treatment modalities including immunosuppressive therapy with mycophenolate mofetil and cyclosporine and three cycles of plasma exchange, and AIHA responded to treatment. This is the third case of AIHA complicating hematopoietic stem cell transplantation reported in Korea. Since AIHA is relatively common after hematopoietic stem cell transplantation, accurate and timely diagnosis of the disease and treatment strategies with multiple modalities are necessary.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Combined Modality Therapy ; Cyclosporine/therapeutic use ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Male ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Myelodysplastic Syndromes/complications/diagnosis/therapy ; Plasma Exchange

OBJECTIVE: To evaluate the efficacy and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with decitabine (Dec)-conditioning regimen in the treatment of myelodysplastic syndrome (MDS) and MDS transformed acute myeloid leukemia (MDS-AML). METHODS: The characteristics and efficacy data of 93 patients with MDS and MDS-AML who received allo-HSCT in our center from April 2013 to November 2021 were retrospectively analyzed. All patients were administered by myeloablative conditioning regimen containing Dec (25 mg/m² /d×3 d). RESULTS: Among the 93 patients, 63 males and 30 females, were diagnosed as MDS（n ＝77）, MDS-AML（n ＝16）. The incidence of I/II grade regimen-related toxicity (RRT) was 39.8%, and III grade RRT was only found in 1 patient (1%). Neutrophil engraftment was successful in 91 (97.8%) patients after a median neutrophil engraftment time of 14 (9-27) days; Successful platelet engraftment was achieved in 87 (93.5%) patients, with a median engraftment time of 18 (9-290) days. The incidence of acute graft versus host disease（aGVHD） and grade III-IV aGVHD was 44.2% and 16.2%, respectively. The incidence of chronic graft versus host disease（cGVHD） and moderate-to-severe cGVHD was 59.5% and 37.1%, respectively. Of the 93 patients, 54 (58%) developed posttransplant infections, among which lung infection (32.3%) and bloodstream infection (12.9%) were the most common. The median follow-up after transplantation was 45 (0.1-108) months. The 5-year overall survival (OS) rate, disease-free survival (DFS) rate, treatment-related mortality, and cumulative incidence of relapse were 72.7%, 68.4%, 25.1%, and 6.5%, respectively. And the 1-year graft-versus-host disease/relapse-free survival rate was 49.3%. The patients in different group of relative high-risk prognostic scoring or low-risk prognostic scoring, with or without poor-risk mutation(s), with mutations number ≥3 or <3 had similar 5-year OS rate (more than 70%). Multivariate analysis showed that the incidence of grade III-IV aGVHD was the independent risk factor affecting OS（P =0.008）and DFS (P =0.019). CONCLUSION Allo-HSCT with Dec-conditioning regimen is feasible and effective in the treatment of patients with MDS and MDS-AML, especially those in high prognostic risk and with poor-risk mutations.
Male ; Female ; Humans ; Decitabine ; Retrospective Studies ; Transplantation, Homologous/adverse effects* ; Transplantation Conditioning/adverse effects* ; Myelodysplastic Syndromes/complications* ; Leukemia, Myeloid, Acute/therapy* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Chronic Disease ; Graft vs Host Disease/therapy* ; Recurrence