Generalized pustular psoriasis is a severe form of paoriasis and is frequently associated with arthritis and nail deformity. We present a case of generalized pustular psoriasis in a 40 year-old man, with nail change, arthritis of both knee and ankle joints, spondylitis, and glomerulonephritis. We found a positive results of HLA A-9, B-27 in this patient. Skin lesions, arthralgia and hematuria improved marked1y by plasmapheresis and application of topical corticostertoid.
Adult ; Ankle Joint ; Arthralgia ; Arthritis* ; Congenital Abnormalities ; Glomerulonephritis* ; Hematuria ; Humans ; Knee ; Plasmapheresis ; Psoriasis* ; Skin ; Spondylitis*

The importance of the ticks in medical field has been emphasized because of their association with and transmission of various diseases. We report herein a case of tick bite in a 55-year-old male farmer, who visited our hospital on July 2, 1981 with a parasite attached on right lower flank and rice to small pea sized, pruritic erythematous papular skin eruptions on chest and right lower flank. The skin lesions disappeared completely with.in five days after removal of the parasite, which was identified with an adult female tick which belongs to Genus Ixodes.
Adult ; Female ; Humans ; Ixodes* ; Male ; Middle Aged ; Parasites ; Peas ; Skin ; Thorax ; Tick Bites* ; Ticks*

PURPOSE: In clinically suspected ankylosing spondylitis of sacroiliac (SIJ) and hip joints with normal or minimal secondary bone change in simple X-ray films, we evaluated the role of MRI in sacroiliac and hip joints. MATERIALS AND METHODS: Authors evaluated 11 cases (36 joints;SIJ 14, hip 22) confirmed as spondylitis by clinical, laboratory, and radiologic findings, and compared the detectability of involvement of joints by simple X-ray film and MRI. Authors analysed MR findings for the presence of pannus and its si, intensities (SI), change of articular cartilage, bony erosion and sclerosis, subchondral bone cysts,.~ osteophytosis, bone marrow edema, joint effusion, adjacent soft tissue change, and contrast enhancement ofi pannus. RESULTS: MRI detected not only 20 joints (SIJ 11, hip 9) detected in simple X-ray, but also additional 7 joints (SIJ 3, hip 4). MRI depicted simultaneous involvement of SIJ and hip joints in 5 of 11 cases (SIJ 10 joints, hip9 joints), and bilateral involvement of SIJ and hip joints in 4 among the 5 cases. MRI also demonstrated pannus, which were not detected in conventional films, as intermediate SI on T1WI and high SI on T2WI, in all 27 joints (SIJ 14, Hip 13). Gd-DTPA enhanced T1WI revealed enhancement of pannus in 7 cases (17 joints). CONCLUSION: MRI was a valuable modality in evaluation of clinically suspected ankylosing spondylitis of SIJ or hip joints with normal or minimal secondary bone change in simple X-ray. Simultaneous evaluation of SIJ and hip joints is suggested in clinically suspected ankylosing spondylitis or other joint diseases.
Bone Marrow ; Cartilage, Articular ; Edema ; Gadolinium DTPA ; Hip Joint* ; Hip* ; Joint Diseases ; Joints ; Magnetic Resonance Imaging ; Sclerosis ; Spondylitis ; Spondylitis, Ankylosing* ; X-Ray Film

The next-generation sequencing (NGS), as the most practical and reliable method, has replaced the classical Sanger sequencing to help scientists to discover the genetics secrets of human tumor diseases. With the technique development, the whole genome sequencing will be no longer out of reach. Recently, some scientists used the NGS in the research of hematological malignancies and pushed the progress of the whole genome sequencing in acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) actively in order to find out the pathogenesis of some hematological malignancies. The NGS and the application of the whole genome sequencing, the exome sequencing, the transcriptome sequencing in AML and MDS are reviewed in this article.
Base Sequence ; Genome, Human ; Hematologic Neoplasms ; genetics ; Humans ; Leukemia, Myeloid, Acute ; genetics ; Myelodysplastic Syndromes ; genetics ; Sequence Analysis ; methods

A case of a 21-year old female with Maffucci's syndrome is presented where the patient developed multiple cutaneous spindle-cell hemangioendotheliomas. She had multiple pea to walnut sized, non-tender, normal skin colored or slight bluish nodules and bony mass-like lesions on the left upper extremity and hand. A histological examination of the lesions showed that they were composed of irregularly dilated, thin walled cavernous blood spaces containing phleboliths and collapsed vascular spaces separated by spindled fibroblastic cells. Radiologically, the bony lesions showed radiolucent densities with calcified spots within the second proximal phalanx and metacarpal bone of the left hand, which were consistent with enchondromas.
Chondroma ; Female ; Fibroblasts ; Hand ; Hemangioendothelioma* ; Humans ; Juglans ; Peas ; Skin Pigmentation ; Upper Extremity

Ineffective erythropoiesis is recognized as the principal reason of non-transfusional iron overload. In the process of expanded erythropoiesis, the apoptosis of erythroblasts induces the up-regulation of GDF15. GDF15 suppresses hepcidin production by the hepatocytes. Subsequently, low hepcidin levels increase iron absorption from the intestine resulting in iron overload. Physiological dose of GDF15 can promote the growth and differentiation of erythroid progenitors, but the high dose of GDF15 can suppress the secretion of hepcidin. The regulation of GDF15 may also be related to iron levels, epigenetic regulation and hypoxia. In this article the GDF15 and its expression and distribution, roles of GDF15 in erythropoiesis and iron overload, as well as the regulation factors of GDF15 are reviewed.
Erythropoiesis ; Growth Differentiation Factor 15 ; metabolism ; Humans ; Iron Overload

Myelodysplastic syndromes (MDS) are hematopoietic malignancies characterized by peripheral cytopenia and dysplastic bone marrow that arise from mutations in the hematopoietic stem/progenitor cells (HSPC). Recently, significant effects have been made to develop appropriate mouse models to study this complex disease. Three general approaches have been used to establish the MDS mouse, including treatment with mutagens or carcinogen, xenotransplantation of human MDS cells, and genetic engineering of mouse hematopoietic cells. In this review, several MDS mouse models and the advances of study on the mechanisms of malignant clone and the marrow microenvironment are summarized. In addition, the progress in xenotransplantation models of MDS and the problems to be solved are discussed briefly.
Animals ; Cellular Microenvironment ; Disease Models, Animal ; Mice ; Myelodysplastic Syndromes

Myelodysplastic syndrome (MDS) is a group of heterogeneous clonal diseases characterized by ineffective hematopoiesis, peripheral blood cytopenias and high risk of transformation to acute myeloid leukemia.Recently more and more investigations indicate that the abnormality of bone marrow microenvironment is one of important reasons related to MDS. In this article the abnormality of stroma cells, cytokines and signaling pathways in hematopoietic micro-environment of MDS is reviewed.
Bone Marrow ; Cellular Microenvironment ; Cytokines ; metabolism ; Humans ; Myelodysplastic Syndromes ; Signal Transduction ; Stromal Cells ; metabolism

Myelodysplastic syndrome (MDS) is clonal disorder of hematopoiesis characterized by inefficient hematopoiesis, peripheral blood cytopenias, aberrant differentiation, and risk of progression to acute myeloid leukemia. Although specific karyotypic abnormalities have been found to link to MDS for decades, more recent findings have demonstrated the importance of mutations within individual genes. The recent molecular abnormalities found in MDS include following gene mutation such as TET2, TP53, RUNX1, ASXL1, IDH1/IDH2, EZH2 and RAS. In this review, the recent advances of prognostic molecular markers of MDS and their biological and clinical significance are summarized.
DNA-Binding Proteins ; genetics ; Humans ; Mutation ; Myelodysplastic Syndromes ; diagnosis ; genetics ; Prognosis ; Proto-Oncogene Proteins ; genetics