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.
Aged ; Biopsy ; Human ; Male ; Myelodysplastic Syndromes/*complications ; Polychondritis, Relapsing/*complications/*diagnosis/pathology ; Red-Cell Aplasia, Pure/*complications/pathology

This study was aimed to investigate the immunologic characteristics of refractory anemia with excess blasts-II (RAEB-II) which belongs to a new subtype of World Health Organization (WHO) classification of myelodysplastic syndrome (MDS) and to screen out the independent immunologic prognostic factors of MDS. 35 cases of adult patients with de novo MDS were investigated. The immunofluorescent analysis by multiparameter flow cytometry was performed at the double gating of CD45/SSC to determine the immunophenotype of MDS cells in all cases. All patients were followed up. 47 cases of acute myeloid leukemia (AML) M1, 51 cases of AML-M(2) and 38 cases of acute lymphocytic leukemia (ALL) were selected as control. Software SPSS 13.0 was applied to analyze all the related data. The results showed that the positive expression rate of HLA-DR in RAEB-II was 100%, which was high in sensitivity and specificity. CD13 (94.74%), CD33 (84.21%) and CD117 (78.95%) were also highly expressed in RAEB-II. CD13 in RAEB-II was significantly higher than that in refractory cytopenia with or without multilineage dysplasia (RA/RCMD) (p < 0.01) and REAB-I (p < 0.05); CD33, CD117 (p < 0.05) and stem cell antigen CD34 (p < 0.01) in RAEB-II were significantly higher than that in RCMD (p < 0.01), but no statistically significant difference was found as compared with RAEB-I (p > 0.05). Compared with AML-M(1) and AML-M(2), no significant difference of CD13 and CD117 in RAEB-II was found (p > 0.05). CD33 (p < 0.01) and CD34 (p < 0.05) were significantly lower than that in AML-M(1), but no significant difference was found as compared with AML-M(2) (p > 0.05); CD15 (p < 0.01) and CD11b (p < 0.05) was significantly lower than that in M(2), but no significant difference was found as compared with AML-M(1) (p > 0.05); MPO was significantly lower than that in AML-M(1) and M(2) (p < 0.05); HLA-DR was significantly higher than that on AML-M(2) (p < 0.05), but no significant difference was found as compared with AML-M(1) (p > 0.05). RAEB-II did not express CD2, CD3, CD5 and CD8 (positive rate 0%, p < 0.01) when compared with T-ALL; CD4 (p < 0.05) and CD7 (p < 0.01) were significantly lower than that in T-ALL. RAEB-II did not express CD19 and CD20 (positive rate 0%, p < 0.01) as compared with B-ALL; CD10, CD22 and cCD79a were significantly lower than that in B-ALL (p < 0.05). CD117 (p = 0.0197) and MPO (p = 0.0085) were the two prognostic immunological antigens as regards the overall survival (OS) of MDS; CD117 (p = 0.003) was the single parameter in Cox regression. It is concluded that RAEB-II expresses mainly myeloid antigen without or with little expression of lymphoid antigen. Unique individual immunophenotypic features can be detected in patients with RAEB-II. HLA-DR can be a specific parameter to distinguish the other subtypes of MDS. CD117 may be an independent prognostic immunological antigen as regards OS of MDS.
Adolescent ; Adult ; Aged ; Anemia, Refractory ; complications ; diagnosis ; immunology ; Blast Crisis ; complications ; diagnosis ; immunology ; Female ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes ; complications ; diagnosis ; immunology ; Prognosis ; Young Adult

No abstract available.
Chromosome Aberrations ; Humans ; Karyotype ; Leukemia, Myeloid, Acute/*diagnosis/etiology/mortality ; Myelodysplastic Syndromes/complications/*diagnosis ; Myeloproliferative Disorders/complications/*diagnosis ; Philadelphia Chromosome ; Survival Rate

OBJECTIVETo explore the value of morphological examination, cytochemical staining combined with bone marrow biopsy in the differential diagnosis between myelodysplastic syndrome (MDS) with low blasts and hemolytic anemia (HA).

METHODSThe clinical data of 85 cases of myelodysplastic syndrome with low blasts (< 5%) and 61 patients with hemolytic anemia in Chinese PLA's Gerneral hospital from September 2009 to March 2015 were retrospectively analysed. The clinical characteristics, cytogenetic and molecular features, bone marrow cell count and morphology features, cytochemical staining results and bone marrow biopsy features of above-methioned patients were compared.

RESULTSThere was no significant difference (P > 0.05) in clinical data between MDS group and HA group. Megakaryocytic dysplasia-positive rate, and ring sideroblasts positive rate, and PAS positive rate were significantly higher in MDS group than those that in HA group (P < 0.05). Abnormal localization of immature precursors (ALIP) and megakaryocytic dysplasia positive rate in bone marrow biopsy were significantly higher in MDS group than those that in HA group (P < 0.05), 90.6% of MDS with low blasts patients were identifiable by combined detections.

CONCLUSIONCombining detection of morphology, cytochemistry staining and bone marrow biopsy has been confirmed to be more useful for differential diagnosis between MDS with low blasts and HA.

Anemia, Hemolytic ; complications ; diagnosis ; Biopsy ; Bone Marrow Cells ; cytology ; Diagnosis, Differential ; Erythroid Precursor Cells ; cytology ; Humans ; Megakaryocytes ; cytology ; Myelodysplastic Syndromes ; complications ; diagnosis ; Retrospective Studies ; Staining and Labeling

Endoscopic submucosal dissection (ESD) has been successfully performed in thrombocytopenic conditions such as in patients with liver cirrhosis but successful ESD for early gastric cancer (EGC) in hematologic diseases has rarely been reported. A 52-year-old male patient, who had previously been diagnosed with myelodysplastic syndrome 2 years ago, was admitted to our hospital for ESD of EGC. ESD was performed successfully in this patient after platelet concentrates transfusion on the day of ESD. ESD might be an option for the treatment of EGC in thrombocytopenia due to hematologic diseases when optimal supportive managements are applied.
Early Detection of Cancer ; Endosonography ; Gastric Mucosa/*surgery ; Gastroscopy ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes/complications/*diagnosis/pathology ; Stomach Neoplasms/complications/*diagnosis/pathology ; Tomography, X-Ray Computed

OBJECTIVETo investigate the prognostic value of thrombocytopenia in patients with primary myelodysplastic syndromes (MDS).

METHODSFour hundred and nineteen primary MDS patients were retrospectively analyzed. Kaplan-Meier method, Log-rank test and COX regression model were used to evaluate factors that influence the prognosis.

RESULTSTwo hundred and fifty-six cases (61.1%) had thrombocytopenia (PLT < 100×10(9)/L), one hundred and three cases (24.6%) had severe thrombocytopenia (PLT < 30×10(9)/L). Overall survival (OS) tended to shorten along with the decreasing of platelet count. Univariate analysis indicated that PL < 30×10(9)/L, MCV ≤ 95 fl, LDH ≥ 300 U/L, lymphocyte-like micromegakaryocyte, nucleated RBC PAS positive, IPSS cytogenetic intermediate- and poor-risk were all related with poor prognosis. Moreover, the prognosis of patients with RCMD, RAEB-Ior RAEB-IIwas poorer than that of the other subgroups. Among these parameters, PLT < 30×10(9)/L, MCV ≤ 95 fl, IPSS cytogenetic intermediate- and poor-risk group and RCMD, RAEB-I and RAEB-II had independent prognostic significance in multivariate analysis. Modified WPSS prognostic model was proposed by adopting PLT, MCV, chromosomal karyotype and WHO classification. The OS of patients with low risk, intermediate-1 risk, intermediate-2 risk and high risk were 59, 28, 14 and 4 months, respectively, and there was a statistically significant difference between the groups (P < 0.05).

CONCLUSIONSevere thrombocytopenia indicated unfavorable prognosis, in combination with MCV, chromosomal karyotype and WHO classification, a modified WPSS prognostic model was proposed and worked well for prognostic indication in patients with MDS.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes ; complications ; diagnosis ; Prognosis ; Retrospective Studies ; Thrombocytopenia ; complications ; diagnosis ; Young Adult

OBJECTIVETo analyze the clinical features and prognosis of the primary myelodysplastic syndrome with myelofibrosis (MDS-MF) patients and to improve the cognition of MDS-MF.

METHODSFour 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.

RESULTS167 (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).

CONCLUSIONThe 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.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Humans ; Karyotyping ; Male ; Middle Aged ; Myelodysplastic Syndromes ; complications ; diagnosis ; pathology ; Primary Myelofibrosis ; complications ; diagnosis ; pathology ; Prognosis ; Retrospective Studies ; Young Adult

ST segment elevation is one of the most important electrocardiographic features that need to be recognised. Although ST segment elevation myocardial infarction is one of the main causes of this abnormality, there are other non-ischaemic causes that are also important. We discuss reversible apical ballooning syndrome or Takotsubo cardiomyopathy, pericarditis and a case of ST segment elevation due to 'early repolarisation pattern'.
Cardiology ; methods ; Coronary Angiography ; methods ; Dyslipidemias ; complications ; Electrocardiography ; methods ; Female ; Humans ; Leukemia, Myeloid, Acute ; complications ; Male ; Middle Aged ; Myelodysplastic Syndromes ; complications ; Myocardial Infarction ; complications ; diagnosis ; Myocardial Ischemia ; pathology ; Prostatic Neoplasms ; complications ; Respiratory Tract Infections ; complications

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

BACKGROUND: AML with myelodysplasia related changes (AML MRC) is known to show a poor prognosis compared with de novo AML, but controversies exist about the prognostic impact of multilineage dysplasia (MLD) among MRC. We investigated the prognostic impact of MLD in AML MRC. METHODS: A total of 357 patients newly diagnosed as AML at Asan Medical Center from January 2001 to December 2005 were analyzed. They were diagnosed and classified as AML with recurrent genetic abnormalities, AML MRC, and AML not otherwise specified (AML NOS). Prognostic markers including overall survival (OS) and event free survival (EFS) were obtained through retrospective analysis of electronic medical records. RESULTS: AML MRC patients showed a lower complete remission (CR) rate (44.7% vs. 64.9%, P=0.002) and shorter OS (297 vs. 561 days, P=0.004) and EFS (229 vs. 374 days, P=0.004) than AML NOS patients. Patients with MLD among AML MRC also showed a lower CR rate (37.7%, P=0.001) and shorter OS (351 days, P=0.036) and EFS (242 days, P=0.076) than AML NOS patients. However, among AML MRC patients, there were no differences in OS, EFS and CR between patients with and without MLD. CONCLUSIONS: AML MRC patients showed a lower CR rate and shorter OS and EFS than AML NOS patients. AML MRC patients with MLD showed similar results and their prognosis was not different from those without MLD. MLD findings among AML MRC could be an independent poor prognostic factor in de novo AML.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Cell Lineage ; Child ; Child, Preschool ; Data Interpretation, Statistical ; Disease-Free Survival ; Female ; Humans ; Infant ; Leukemia, Myeloid, Acute/complications/diagnosis/*mortality ; Male ; Middle Aged ; Myelodysplastic Syndromes/complications/*diagnosis ; Prognosis ; Retrospective Studies ; Survival Analysis