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A Case of Catastrophic Primary Antiphospholipid Syndrome.

Joon Hyung DOH ; Jung Yoon CHOE ; Jae Hoon KIM ; Chang Soo KIM ; Dong Ho OH ; Byung Reul CHOI ; Jae Kean RYU ; Dae Sung HYUN ; Ho Gak KIM ; Yong Jin KIM ; Sang Gyung KIM

Korean Journal of Medicine.1999;56(2):240-246.

Catastrophic antiphospholipid syndrome is a rare clinical syndrome characterized by acute multi-organ failure occurring in patients with antiphospholipid antibodies. It is associated with involvement of several end-organs particularly kidneys, lungs, gastrointestinal tracts and adrenal glands and presents catastrophic clinical pictures such as acute renal failure with thrombotic microangiopathy, myocardial failure, adult respiratory distress syndrome, convulsion and disseminated intravascular coagulation. Conventional treatments(e.g. intravenous heparin, steroid, immunosuppressants) were not effective, while plasmapheresis seems to be a useful therapy. We experienced a case of catastrophic primary antiphospholipid syndrome in 41-year-old woman proved by renal biopsy and immuno-serological tests. She developed acute renal failure, multiple esophageal and oral ulcers, adult respiratory distress syndrome, abnormal elevation of hepatic and pancreatic enzymes and signs of disseminated intravascular coagulation. Evidences of any other connective tissue diseases were not found. Renal biopsy revealed features of thrombotic microangiopathic nephropathy and serum antiphospholipid antibody level was elevated(34GPL). In spite of steroid, cyclophosphamide and supportive therapies, her respiratory distress was not improved.
Acute Kidney Injury ; Adrenal Glands ; Adult ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Biopsy ; Connective Tissue Diseases ; Cyclophosphamide ; Disseminated Intravascular Coagulation ; Female ; Gastrointestinal Tract ; Heart Failure ; Heparin ; Humans ; Kidney ; Lung ; Oral Ulcer ; Plasmapheresis ; Respiratory Distress Syndrome, Adult ; Seizures ; Thrombotic Microangiopathies

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A Case of CD5 Negative B-Cell Chronic Lymphocytic Leukemia.

Oh Kil KIM ; Jin Suk CHEON ; Hag Jun KIM ; Gun Ho LEE ; Yong Jun AN ; Joon Ho HA ; Kyung Seok OH ; Sang Ryong LEE ; Hyung KIM ; In Sun JUN ; Myeong You KIM ; Jeong Nyeo LEE

Korean Journal of Medicine.1999;56(2):235-239.

A 67-year-old male visited Pusan Veterans Hospital due to general weakness and weight loss for 6 months. Physical examination showed non-tender 4 finger breaths sized splenomegaly and both inguinal and cervical lymphadenopathy. The white blood cell count was 25,300/uL with 91% morphologically mature lymphocytes. Bone marrow aspirate revealed hypercellularity with 74.5% lymphocytes morphologically similar to peripheral lymphocytes. The immunophenotpying study of lymphocytes displayed the phenotype of CD19(+), CD20(+), HLA-DR(+), sIg(+) but CD5(-). We concluded that this patients's diagnosis is CD 5 negative B-cell chronic lymphocytic leukemia.
Aged ; B-Lymphocytes* ; Bone Marrow ; Busan ; Diagnosis ; Fingers ; Hospitals, Veterans ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell* ; Leukocyte Count ; Lymphatic Diseases ; Lymphocytes ; Male ; Phenotype ; Physical Examination ; Splenomegaly ; Weight Loss

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Solitary Plasmacytoma of Iliac Bone Rapidly Progressed to Multiple Myeloma.

Sang Mi YUN ; Sang Il KIM ; Hong Suk SONG

Korean Journal of Medicine.1999;56(2):229-234.

Solitary osseous plasmacytoma accounts for 3-5% of plasma cell tumor and are assumed to have a fairly good prognosis, with long duration of relapse free survival after local irradiation. A 64 year old woman with a lytic lesion involving left iliac bone was diagnosed as a solitary plasmacytoma, with a negative work-up for coexisting plasma cell disorders. Three months after irradiation of 5,400 cGy, the patient was readmitted with hypercalcemia and mass in left forehead and left gingiva. New multiple osteolytic lesions were developed. A aspiration cytology of forehead mass and curetted specimen of right femur due to pathologic fracture revealed plasmacytoma. MRI of thoracolumbar spine revealed abnormal high signal intensity in the L2 body, T8 and T9 vertebrae. Serum protein electropheresis revealed monoclonal gammopathy of IgG-kappa type. This aggressive case of solitary plasmacytoma, evolving into multiple myeloma after brief duration of remission, is in sharp contrast with the natural course of a solitary plasmacytoma.
Female ; Femur ; Forehead ; Fractures, Spontaneous ; Gingiva ; Humans ; Hypercalcemia ; Magnetic Resonance Imaging ; Middle Aged ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells ; Plasmacytoma* ; Prognosis ; Radiotherapy ; Recurrence ; Spine

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A Case Report of Purple Toe Syndrome Associated with Acute Renal Failure during Warfarin Therapy.

Dong Ryeol RYU ; Jae Hoon LEE ; Hyo Kyoung PARK ; Young Jun CHO ; Jeong Ho CHO ; Sung Kwan HONG ; Shin Wook KANG ; Kyu Hun CHOI ; Seung Yun CHO ; Dae Suk HAN ; Ho Yung LEE

Korean Journal of Medicine.1999;56(2):225-228.

"Purple toe" syndrome is an extremely rare complication of warfarin therapy. The occurrence of purple toe syndrome is characterized by a sudden appearance of purplish discoloration of toes and the sides of feet. The skin lesions usually develop 3-8 weeks after beginning anticoagulation. The pathogenesis was not clearly defined but the presence of atherosclerosis in most of patients led to suggest that the mechanism was related to cholesterol emboli from the atherosclerotic plaques and warfarin- induced bleeding into the plaques. These microemboli are commonly associated with irreversible organ dysfunction such as renal failure, distal gangrene, pancreatitis, and multifocal myocardial necrosis. Therefore purple toe syndrome may be considered as a sentinel of cholesterol crystal embolism. Once established, anticoagulation and thrombolysis are contraindicated. Necrosis and gangrenous changes may result in loss of limb and occasional mortality has been reported. We report a case of purple toe syndrome associated with acute renal failure after warfarin therapy with a review of literatures.
Acute Kidney Injury* ; Atherosclerosis ; Cholesterol ; Embolism ; Embolism, Cholesterol ; Extremities ; Foot ; Gangrene ; Hemorrhage ; Humans ; Mortality ; Necrosis ; Pancreatitis ; Plaque, Atherosclerotic ; Renal Insufficiency ; Skin ; Toes* ; Warfarin*

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A Case of Transient Hyperkalemia and Hyperaldosteronism secondary to Hydronephrosis.

Sung Bae LEE ; Jung Ho UHM ; Keun Man LEE ; Kil Sang WANG ; Young Hoon KIM ; Kyung Ah HAN ; Kyung Wan MIN ; Seol Hye HAN ; Eung Jin KIM

Korean Journal of Medicine.1999;56(2):220-224.

Renal tubular dysfunctions have been observed in hydronephrosis, resulting in metabolic acidosis, hyperkalemia and excessive free water diuresis. These findings are occasionally found in infant and children. Batle et al. first reported distal tubular acidosis associated with low potassium excretion resulting from aldosterone resistance in adults with obstructive uropathy. We have experienced a case of transient hyperkalemia and hyperaldosteronism secondary to hydronephrosis in 63-year-old female patient. The causes of hyperkalemia were examined under the impression of secondary adrenal insufficiency due to corticosteroid abuse or hyporeninemic hypoaldosteronism due to diabetic nephropathy. But it proved to be resulted from hyperaldosteronism due to hydronephrosis. The hyperkalemia resulting from hyperaldosteronism is rare in adults. It may result from aldosterone resistance at distal nephron due to obstructive uropathy or the defect of distal nephron in hydrogen and potassium secretion in the distal nephron rather than from aldosterone deficiency. After she underwent percutaneous nephrostomy, serum potassium was maintained within normal range. She performed total cystectomy with ureterocutaneostomy in purpose for treatment of bladder cancer. So we report this case with a review of literature.
Acidosis ; Adrenal Insufficiency ; Adult ; Aldosterone ; Child ; Cystectomy ; Diabetic Nephropathies ; Diuresis ; Female ; Humans ; Hydrogen ; Hydronephrosis* ; Hyperaldosteronism* ; Hyperkalemia* ; Hypoaldosteronism ; Infant ; Middle Aged ; Nephrons ; Nephrostomy, Percutaneous ; Potassium ; Reference Values ; Urinary Bladder Neoplasms ; Water

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A Case of Respiratory Bronchiolitis-Associated Interstitial Lung Disease.

Young Ju PARK ; Jae Hwa CHO ; Jeong Seon RYU ; Hong Lyeol LEE

Korean Journal of Medicine.1999;56(2):215-219.

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is an inflammatory lung disorder associated with cigarette smoking. The clinical and radiographic findings of RB-ILD are nonspecific and most patients with RB-ILD are thought to have some form of idiopathic pulmonary fibrosis prior to lung biopsy. Open lung biopsy of patients with RB-ILD reveals inflammation of the respiratory bronchioles, filling of the bronchiolar lumens and surrounding alveoli with finely pigmented macrophages, associated interstitial inflammation. Alveolar septa, particularly around terminal and membranous bronchioles, are mildly fibrotic. We recently experienced a case of RB-ILD proven by open lung biopsy.
Biopsy ; Bronchioles ; Humans ; Idiopathic Pulmonary Fibrosis ; Inflammation ; Lung ; Lung Diseases, Interstitial* ; Macrophages ; Smoking

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Delayed diagnosis of an endobronchial foreign body confused with bronchial asthma.

Byung Joo SUN ; Ji Young CHOI ; Yun Jeong BAE ; Chan Sun PARK ; Tae Bum KIM ; You Sook CHO ; Hee Bom MOON

Korean Journal of Medicine.2009;76(2):244-247.

Tracheobronchial foreign bodies can remain undetected for months, or even years, and often present as chronic respiratory symptoms, such as an intractable cough. We report the case of a 51-year-old woman with a cough for over 3 years and hemoptysis for 2 weeks. She had been treated for asthma for the previous 3 years because of her cough, wheeze, and positive bronchodilator response. Her symptoms waxed and waned. Her chest X-ray showed a new mass-like opacity and ill-defined infiltration in the right lower lobe. Computed tomography showed a 2.5-cm mass-like lesion in the right infrahilar area. At fiberoptic bronchoscopic, a solid foreign body was found in the right lower lobe bronchus and was identified as a fish bone. Tracheobronchial foreign body aspiration should always be considered in the differential diagnoses of radiographic lesions or chronic respiratory symptoms that cannot be easily explained.
Asthma ; Bronchi ; Cough ; Delayed Diagnosis ; Diagnosis, Differential ; Female ; Foreign Bodies ; Hemoptysis ; Humans ; Middle Aged ; Thorax

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Diagnosis and management of primary malignant melanoma of the lung: a case report.

Chang Seok BANG ; Geun Woo PARK ; Soon Il LEE ; Young Kwang CHOO ; Won Kyung LEE ; Ran NOH ; Ji Hyun SUH

Korean Journal of Medicine.2009;76(2):238-243.

Melanoma develops in the skin or the mucosa of organs. It spreads via lymphatic channels or the bloodstream to the regional lymph nodes or organs such as the brain, liver, and lung. Melanoma of the lung is nearly always metastatic, and primary malignant melanoma of the lung is very rare, with only 30 cases reported in the English literature. The possibility of skin lesions that have disappeared, or of undetected primary sites, makes it difficult to confirm true primary tumors. Therefore, a thorough workup is needed to make the diagnosis. Since metastatic melanoma is incurable, the goal of treatment is palliation. However, because of the rare experience of primary melanoma of the lung, it is difficult to predict the prognosis. We experienced one patient with primary malignant melanoma of the lung with metastases who had been managed with surgery, interferon-alpha, chemotherapy, and radiation therapy over 5 years. This case report presents the diagnosis and management of primary malignant melanoma of the lung.
Brain ; Humans ; Interferon-alpha ; Liver ; Lung ; Lung Neoplasms ; Lymph Nodes ; Melanoma ; Mucous Membrane ; Neoplasm Metastasis ; Prognosis ; Skin

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An intrathoracic malignant peripheral nerve sheath tumor in a neurofibromatosis type 1 patient.

Jin Yi CHOI ; Jeong Hun KIM ; Ja Sung CHOI ; Jae Ho CHUNG ; Jeong Eun CHOI ; Hwa Eun OH ; Chan Sub PARK

Korean Journal of Medicine.2009;76(2):234-237.

A malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue tumor defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. It is one of the malignant tumors associated with neurofibromatosis type 1 (von Recklinghausen's disease). They occur most commonly on the lower and upper extremities, trunk, head, and neck, while intrathoracic tumors are very rare. We report an intrathoracic MPNST in a 66-year-old female with neurofibromatosis type 1.
Aged ; Female ; Head ; Humans ; Neck ; Nerve Sheath Neoplasms ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nerves ; Upper Extremity

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A case of spontaneous ureteral rupture in a patient on hemodialysis.

Pyung Chun OH ; Young Sil EOM ; Jae Chan PARK ; Woo Jin HAN ; Ju Young SUNG ; Hyun Hee LEE ; Woo Kyung CHUNG

Korean Journal of Medicine.2009;76(2):229-233.

Spontaneous ureteral rupture is rare, and refers to leakage in the absence of prior ureteral manipulation, external trauma, previous surgery, or any destructive kidney disease. It presents a major diagnostic challenge due to the diversity at presentation. Here, we present a rare case of spontaneous ureteral rupture in a 62-year-old man with a history of fungal pyonephrosis (Candida) on maintenance hemodialysis, causing a large infected urinoma and abscess and a review the literature.
Abscess ; Humans ; Kidney Diseases ; Middle Aged ; Pyonephrosis ; Renal Dialysis ; Rupture ; Ureter ; Urinoma

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