Osteosclerotic myeloma is a rare entity, characterized by single or multiple osteosclerotic bone lesions and usually accompanied by a polyneuropathy syndrome (POEMS). Multiple myeloma with osteosclerotic lesions without polyneuropathy is exceedingly rare. We report a case of multiple myeloma associated with multifocal osteosclerotic lesions without any evidence of POEMS. A 48-year-old woman presented with incidentally found osteosclerosis of 8th thoracic vertebra on a plain chest film. Bone survey, CT scan, MR scan, and radioisotope scintigraphy revealed multiple localized osteoclerosis; serum protein immunofixation showed IgG, lambda monoclonal gammopathy. A biopsy of T8 vertebral body disclosed plasma cell myeloma. Given that there was no organ or tissue damage other than multifocal osteosclerosis, the patient was placed on close observation with regular examination. This case indicates that although rare, multiple myeloma should be included in the differential diagnosis of sclerotic bone lesions.
Biopsy ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G ; Middle Aged ; Multiple Myeloma ; Osteosclerosis ; Paraproteinemias ; Polyneuropathies ; Spine ; Thorax

Multiple myeloma is a malignant disease of plasma cells, whereas ankylosing spondylitis is a chronic inflammatory disease of axial joints. The relationship between the two diseases is uncertain, but chronic inflammation could trigger multiple myeloma. The authors report the cases of two ankylosing spondylitis patients with a disease duration of more than 20 years, that subsequently developed IgA kappa and IgG lambda chain myeloma, respectively, and discuss the possible pathogenetic relationship between these diseases.
Humans ; Immunoglobulin A ; Immunoglobulin G ; Inflammation ; Joints ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Plasma Cells ; Spondylitis, Ankylosing

Reversible posterior leukoencephalopathy syndrome (RPLS) is a distinctive clinicoradiological entity that's characterized by headache, confusion, seizure and frequent visual disturbances. It is associated with certain neuro-radiological findings, and predominantly white matter abnormalities of the parieto-occipital lobes. RPLS has been identified mostly in patients with malignant hypertension, pre-eclampsia and renal insufficiency and in those patients who are using immunosuppressive agents or cytotoxic drugs. We report here on a case of RPLS in a patient who was undergoing chemotherapy. A 49-year-old woman presented with abrupt mental changes and visual disturbances five days after the administration of a chemotherapeutic agent. MRI showed hyper-intense signals on the magnetic resonance (MR) diffusion images in the bilateral temporal, parietal and occipital lobes. The clinical manifestations completely resolved after one week of treatment that consisted of blood pressure control, a negative intake-output balance and the best supportive care. These radiological changes and the reversible clinical manifestations were consistent with RPLS.
Antineoplastic Combined Chemotherapy Protocols ; Blood Pressure ; Cisplatin ; Cytarabine ; Diffusion ; Etoposide ; Female ; Headache ; Hodgkin Disease ; Humans ; Hypertension, Malignant ; Immunosuppressive Agents ; Magnetic Resonance Spectroscopy ; Middle Aged ; Occipital Lobe ; Posterior Leukoencephalopathy Syndrome ; Pre-Eclampsia ; Prednisone ; Renal Insufficiency ; Seizures

Castleman's disease (CD) is an uncommon lymphoproliferative disorder presenting with a variable spectrum of clinical manifestations. CD is classified into two subtypes, Localized CD and Multicentric CD, by clinical manifestation, and is divided into 2 histopathologic types: a hyaline-vascular type (HV) or a plasma-cell type (PC). Multicentric CD show PC type predominantly and show systemic symptoms, such as fever, night sweat, malaise, ascites, edema, and more than half show multiple lymphadenopathy and hepatosplenomegaly. We report a case of 67 year old man with multicentric CD presented with fever of unknown origin and diagnosed with lymph node biopsy and bone marrow examination with a brief review of the literature.
Ascites ; Biopsy ; Bone Marrow ; Bone Marrow Examination ; Edema ; Fever ; Fever of Unknown Origin ; Giant Lymph Node Hyperplasia ; Lymph Nodes ; Lymphatic Diseases ; Lymphoproliferative Disorders ; Sweat

Hantavirus is known to a causative agent of hemorrhagic fever with renal syndrome and it can cause hantavirus pulmonary syndrome, a rare disease in Korea. Hantavirus pulmonary syndrome is usually associated with pulmonary hemorrhage and fever. The common hematologic features of this syndrome are elevated hematocrit level, leukocytosis and thrombocytopenia. We report a case of hantavirus pulmonary syndrome with pancytopenia. This patient with severe neutropenia with pulmonary infiltration mimicked aplastic anemia with invasive aspergillosis.
Anemia ; Anemia, Aplastic ; Aspergillosis ; Fever ; Hantavirus ; Hantavirus Pulmonary Syndrome ; Hematocrit ; Hemorrhage ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Korea ; Leukocytosis ; Neutropenia ; Pancytopenia ; Rare Diseases ; Thrombocytopenia

Acquired hemophilia is a rare but potentially life-threatening hemorrhagic disorder caused by the development of autoantibodies against coagulation factor VIII. Concentrates of human factor VIII, desmopressin, activated prothrombin complex concentrates, recombinant activated factor VII can all be used to control episodes of acute bleeding. The recent availability of bypassing agents like recombinant activated factor VII has been shown to be clinically safe and effective as treatment for acute bleeding. In this case report, a 67 year-old male patient with Rh negative blood type developed gross hematuria and bleeding after transurethral resection due to prostatic hypertrophy. After vesicocutaneous fistular reduction operation, post-operative bleeding was presented. The acute bleeding was controlled successfully by the combined treatment with recombinant activated factor VII (Novo seven(R)) and prednisone.
Autoantibodies ; Blood Coagulation Factors ; Deamino Arginine Vasopressin ; Factor VIIa ; Factor VIII ; Hematuria ; Hemophilia A ; Hemorrhage ; Hemorrhagic Disorders ; Humans ; Male ; Prostatic Hyperplasia ; Prothrombin

Myelofibrosis is usually observed in association with hematologic malignancies or metastatic solid tumors, but it has rarely been reported in patients who suffer with autoimmune disorders. Autoimmune myelofibrosis is a distinct clinicopathologic entity and it can occur alone or in association with autoimmune disorders, and the final result is chronic peripheral cytopenia. Primary autoimmune myelofibrosis, in which the autoimmune myelofibrosis is not preceded by a well-defined autoimmune disease, has recently been defined as a distinct clinicopathologic syndrome. We report here on a case of an 18-year-old woman who was diagnosed with primary autoimmune myelofibrosis, and she manifested peripheral pancytopenia, positivity for autoantibodies and Grade III myelofibrosis without having any preceding autoimmune or hematologic disorders.
Adolescent ; Autoantibodies ; Autoimmune Diseases ; Female ; Hematologic Neoplasms ; Humans ; Pancytopenia ; Primary Myelofibrosis

Beta Thalassemia is a very rare disease in Korea. Only14 mutations have been reported in South Korea to date. This is the first case of beta thalassemia intermedia due to hemoglobin Cagliari in Korea. The locus of this missense mutation is exon 3 of beta globin (beta60 Val->Glu). This is considered a de novo mutation of dominantly inherited beta thalassemia in patients previously having no family history of this conditions. The patient showed microcytic hypochromic anemia, jaundice, and splenomegaly, which were typical characteristics of beta-thalassemia intermedia. During follow-up, there was an episode of aplastic crisis due to Parvovirus B19 infection treated with intravenous immunoglobulin. We report this case with the related literature.
Anemia, Hypochromic ; beta-Globins ; beta-Thalassemia ; Exons ; Follow-Up Studies ; Hemoglobins ; Humans ; Immunoglobulins ; Jaundice ; Korea ; Mutation, Missense ; Parvovirus ; Rare Diseases ; Republic of Korea ; Splenomegaly ; Thalassemia

Myeloid sarcoma (MS) is a solid extramedullary tumor that is composed of immature myeloid cells. The incidence of MS in patients with acute myeloid leukemia (AML) has been reported to be 3~8%. Lymph nodes are the most frequent site of presentation for MS, and other clinical presentations such as an abdominal mass with bowel involvement, a pancreatic mass, isolated central nervous system infiltration, a testicular mass and breast lumps have also been reported. AML with mediastinal MS is a relatively rare disease. The patients with AML with mediastinal MS has been reported to have complex cytogenetic abnormalities and a poor long-term prognosis. The optimal treatment of AML with MS remains to be determined. We report here on an unusual case of a 37-year-old man who presented multiple lymph node enlargements on the neck and a huge mediastinal mass that was considered to be lymphoma. He was finally diagnosed as AML with MS according to the surgical excisional biopsy of a left supraclavicular lymph node and bone marrow examination. He achieved complete remission and the multiple enlarged lymph nodes on the neck and the mediastinal mass disappeared after remission induction chemotherapy. Thereafter, he received HLA-matched unrelated allogenic hematopoietic stem cell transplantation.
Adult ; Biopsy ; Bone Marrow Examination ; Breast ; Central Nervous System ; Chromosome Aberrations ; Hematopoietic Stem Cells ; Humans ; Incidence ; Leukemia, Myeloid, Acute ; Lymph Nodes ; Lymphoma ; Mediastinum ; Myeloid Cells ; Neck ; Prognosis ; Rare Diseases ; Remission Induction ; Sarcoma ; Sarcoma, Myeloid

Behcet's disease is a relapsing inflammatory disorder characterized by vasculitis of unknown cause and has been reported rarely in association with malignant diseases. In most cases the autoimmune nature of Behcet's disease and the long-term immunosuppressive therapy for disease control have been proposed to be responsible for malignant transformation. Although a few cases of various solid tumor and myelodysplastic syndrome have been reported in association with Behcet's disease, acute leukemia has seldom been associated with Beh?et's disease in Korea. We report a case of 38-year-old man with acute myeloblastic leukemia association with a Behcet's disease who had not received long-term treatment.
Acute Disease ; Adult ; Humans ; Korea ; Leukemia ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes ; Vasculitis