Manejo de pacientes con síndrome mielodisplásico en época COVID-19 / Management of patients with Myelodysplastic Syndrome in the COVID-19 era

Resumen La infección por coronavirus 2 del síndrome respiratorio agudo grave ha provocado un cambio en la forma de atender a los pacientes con enfermedades hematológicas en todo el mundo. Los pacientes con síndrome mielodisplásico (SMD) se han visto afectados por la ausencia de conocimiento del comportamiento de la enfermedad por coronavirus 2019 (COVID-19) en este tipo de padecimiento. Se han establecido lineamientos internacionales que han permitido continuar con la atención de dichos pacientes. El principal objetivo de esta revisión es definir las medidas preventivas y las estrategias de tratamiento que se deben de tomar al momento de evaluar a un paciente con SMD en la época COVID-19.

Abstract SARS-CoV-2 infection has caused a change in the way we care for patients with hematological diseases around the world. Patients with myelodysplastic syndrome (MDS) have been affected by the lack of knowledge of the behavior of COVID-19 in this type of condition. International guidelines have been established that have made it possible to continue caring for these patients. The main objective of this review is to define the preventive measures and treatment strategies that should be taken when evaluating a patient with myelodysplastic syndrome in the COVID-19 era.

A Case of Relapsing Polychondritis Associated with Myelodysplastic Syndrome with Erythroid Hypoplasia/Aplasia

Relapsing polychondritis (RP) is a rare multisystem disorder. Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia is a rare form of myelodysplasia. Several cases of RP associated with MDS have recently been described. However, RP associated with MDS with erythroid hypoplasia/aplasia has never been reported. There was only one case report of polymyalgia rheumatica associated with MDS with erythroid hypoplasia/aplasia. In this study, we report a 79-year-old patient with RP, who developed MDS subtype refractory anemia (RA) with erythroid hypoplasia/aplasia, a very characteristic subtype of MDS.

Short-term Results of Hematopoietic Stem Cell Transplantation for Children with Myelodysplastic Syndrome

PURPOSE: In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. METHODS: Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA- matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. RESULTS: Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. CONCLUSION: HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.

Mordern treatment of myelodysplastic syndrome / 中国实用内科杂志

Myelodysplastic syndrome (MDS) is a clonal disorders characterized by ineffective hematopoiesis,which can lead to either fatal cytopenias or acute myelogenous leukemias (AML).MDS mainly occurs in the elderly population.The current use of allogeneic hematopoietic stem cell transplantation for MDS patients is the only medical care,but not available for patients who are above 60 years.Recently,several new biological and chemotherapeutic agents have been used in the treatment of MDS alone or jointlly,which may result in the improvement of hematologic parameters and the prevention of disease progression.

Analysis of 5 patients with myelodysplastic syndromes complicated with autoimmune disorders / 中国实用内科杂志

Objective To explore the character of the myelodysplastic syndrome(MDS) with autoimmune disorders (AID).Methods The clinical data of 60 MDS patients were reviewed.Results 5 of 60 MDS patients had AID ,of which 3 had Graves' disease.The commonest MDS was refractory anemia with ringed sideroblasts (RAS).Conclusion MDS might occur with Graves' disease.Humoral immunological abnormality might play an important role in MDS patients with autoimmune disorders and FAB types might not be associated with this.

A case of myelodysplastic syndrome after oral methotrexate overdose / 대한임상병리학회지

Methotrexate is a very potent inhibitor of dihydrofolate reductase and causes bone marrow suppression and megaloblastic anemia. It is widely used in combination with other chemotherapeutic agents in lymphoproliferative disorders. A 63 year old man with ischioneuralgia developed exertional dyspnea and dizziness after he had intentionally taken methotrexate in doses of 5mg per day for 2months. Five months after discontinuation of methotrexate, his bone marrow showed the hypercellular marrow with 90% cellularity, 15% blasts and marked dysgranulopoiesis, suggestive of refractory anemia with excess blasts(RAEB). The hematopoietic cells were not enough aspirated for proper diagnosis in follow up bone marrow after three months. The bone marrow aspirates showed 13% blasts, and marked dysgranulopoiesis. The bone marrow biopsy showed hypercellular marrow with 100% cellularity, but marked fibrosis was developed. The cytogenetic study revealed normal karyotype.

Two Cases of Leukemia Cutis / 대한피부과학회지

We report herein two cases of leukemia cutis. One case is a 54-year-old woman who came to our department with complaints of a solitary ulcerating nodule on her left leg that had been present for 2 months since prior to her visit. Through histopathological studies, the diagnosis of myelocytic leukemia cutis was made before the final diagnosis of acute myelocytic leukemia was made by hematological studies. When combined chemotherapy was finished, she was in a partial remission state and the nodule disappeared after 1 month of chemotherapy. The other case is a 77-year-old man having multiple infiltrative nodules on the right forearm and right thigh for 1 month prior his visit. He was diagnosed as having leukemia cutis for his skin lesions histopathologically. This was redefined as chronic myelomonocytic leukemia of the myelodysplastic syndrome with blastic transfor- mation by hematological examination. He developed septicemia and died 3 weeks after the dermato- logical diagnosis.

A Clinical Observation of Childhood Myelodysplastic Syndrome / 대한소아혈액종양학회지

BACKGROUND: Childhood myelodysplastic syndrome(MDS) is a heterogenous disease complex which has characteristics of cytopenia in one or more hemopoietic cell lines in peripheral blood and of dysmorphisms of hemopoietic precursors in bone marrow, but quite different from adult one. We experienced 22 patients with childhood myelodysplastic syndrome during 9 years from 1987 to 1995 in the Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Pusan, Korea and performed a clinical study about them to analyze the clinical and hematological features and treatment outcome retrospectively. METHOD: We analyzed their clinical features including age and sex distribution, chief complaints and physical findings on first admission, distribution of subtypes, hematologic features including initial hemoglobin, total WBC and platelet count, and treatment outcome including leukmic transformation and prognostic scores. RESULTS: 1) Of the total 22 patients, 10 were in 0-4 years of age on first admission, 8 in 5-9, and 4 above 10 years of age. The male : female sex ratio was 2.1 : 1. 2) Of the 22 patients, chief complaints on first admission were bleeding tendency in 9 patients(40.9%), pallor in 8(36.4%) and fever in 3(13.6%), in order, and physical findings on first admission were purpura or ecchymoses in 17(77.3%), anemia in 15(68.2%) and hepatosplenomegaly in 8(36.4%), in order. 3) Hematologic findings on first admission were as follows : hemoglobin levels were below 3 g/dl in 1 patient(4.5%), 3-6 g/dl in 10(45.5%), and 6-9 g/dl in 11(50.0%). Initial WBC counts were below 5,000/mm3 in 13 patients(59.1%), 5,000-10,000/mm3 in 5(22.7%) and above 10,000/mm3 in 4(18.2%). Initial platelet counts were below 20,000/mm3 in 10(45.5%), 20,000-50,000/mm3 in 5(22.7%), 50,000-100,000/mm3 in 5(22.7%) and above 100,000/mm3 in 2(9.1%). 4) Of the 22 patients, 12 patients(54.6%) were RA type, 1(4.6%) RAS, and 3(13.6%) RAEB, RAEB-T and JCML types, respectively. 5) According to prognostic scores by Mufti et al(1986), none were in 'good' group, 17 patients(89.5%) in 'intermediate' group with 39.5 months of mean duration of survival(range 4-95 months) and 2(10.5%) in 'poor' group with 18 months of mean duration of surviral(range 17-19) until the last follow-up. However, the subtypes and clinical status seemed not to be related to the prognostic scores. 6) Sixteen patients were treated with low dose cytosine arabinoside(10 mg/m2/12hrs), of whom 7 patients gained long-standing event-free survival, whose treatment regimen was changed to oral 6-TG about 2 years later. All 3 of JCML were treated with A-Triple-V regimen, one of whom was died of sepsis, one was transformed into AML and died of sepsis, while the remained one gained long-standig event-free survival (62 months). 7) Leukemic transformation into AML occured in 7 patients(RA 1, RAEB 2, RAEB-T 3, JCML 1), 6 of whom were dead, while one gained long-standing event-free survival of 34 months. CONCLUSION: We concluded that RA was the most dominant type among our patients, and the frequency to transform into AML was 31,8%, and 31.9% of the patients had long-term survival, and that reliability of prognostic scoring system by Mufti et al(1986) was not high.