Peripheral polyneuropathy is caused by various disorders such as diabetes mellitus. Cryoglobulinemia, as a cause of peripheral polyneuropathy, has been well documented in many reports. Recently we experienced a case of essential cyroglobulinemic polyneuropathy and therapeutic effect of plasmapheresis was shown even in chronic periods. In a patient with peripheral polyneuropathy with no known etiology, one should always consider cryoglobulinemia as a cause since early diagnosis and proper treatment will lead to better outcome.
Cryoglobulinemia ; Diabetes Mellitus ; Early Diagnosis ; Humans ; Plasmapheresis ; Polyneuropathies

Cryoglobulinemia ; physiopathology ; Humans ; Male ; Middle Aged ; Peripheral Nervous System Diseases ; diagnosis ; etiology ; Serologic Tests ; Syphilis ; diagnosis

OBJECTIVETo summarize the clinical features of cryoglobulinemia.

METHODWe retrospectively analyzed the clinical data of 30 patients admitted to Peking Union Medical College Hospital from January 2003 to March 2013 due to cryoglobulinemia.

RESULTSThe average age was(53.8±11.9)years in these 30 patients(12 men and 18 women),among whom 22 patient(73.3%)developed infectious diseases including hepatitis B(n=11)and hepatitis C(n=11);in addition,3 hepatitis B patients and 1 hepatitis C patient also had malignancies. Four patients(13.3%)were accompanied with malignant lymphocytic proliferation diseases,and three(10.0%)with connective tissue diseases. The cause of disease was unclear in 5 patients(16.7%). The clinical manifestations varied due to the primary diseases;notably,20 patients(66.7%)had an onset of purpura rash,22(73.3%)and 19(63.3%)were accompanied with hypertension and chronic renal insufficiency,respectively. The severity of renal involvement was relevant with the increase of C reactive protein,erythrocytes,sedimentation rate,and IgM and the decrease of complements. Treatment should be directed at the primary diseases. Glucocorticoid and immunosuppressants were good choices for relieving renal involvement. Elderly, type 1 cryoglobulinemia,and poor renal function were associated with the poor prognosis.

CONCLUSIONSCryoglobulinemia is mainly seen in middle and elderly patients. It can often affect multiple systems,in particular the kidney. Inflammatory markers,IgM,and complements is related with the disease severity. Age,primary disease,and renal function are related with prognosis.

Aged ; Cryoglobulinemia ; diagnosis ; drug therapy ; pathology ; Female ; Hepatitis C ; diagnosis ; Humans ; Immunosuppressive Agents ; therapeutic use ; Kidney ; pathology ; Male ; Middle Aged ; Prognosis ; Retrospective Studies

BACKGROUNDChronic hepatitis C virus (HCV) infection can affect multiple organ systems and cause a variety of extrahepatic manifestations (EMs). We sought to assess the constituent ratio of EMs in Chinese patients with chronic HCV infection and identify the clinical and biological factors associated with EM.

METHODSThe medical records of 297 patients with chronic HCV infection were analyzed and demographic and epidemiological information was collected. The diagnosis of chronic HCV infection was based on positive anti-HCV combined with a positive HCV-RNA or at least two times of elevated aminotransferases attributable to HCV infection. Patients with HBV and/or HIV coinfection, autoimmune hepatitis, and history of alcohol abuse were excluded.

RESULTSSixty-two percent (184/297) of the patients had at least one EM, including fatigue (29.4%), type 2 diabetes mellitus (28.2%), renal involvement (12.5%), lymphadenopathy (9.6%), fever (9.4%), thyroid dysfunction (8.1%), and arthralgia (7.4%). Neuropathy, sicca syndrome, B-cell lymphoma, Raynaud's phenomenon, and lichen planus were rare. The mean age of patients with EM was older compared with those without EM.

CONCLUSIONSEMs were common in Chinese patients with chronic HCV infection, particularly fatigue, type 2 diabetes, renal impairment, lymphadenophy, fever, and thyroid dysfunction. Older age was associated with EMs.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; China ; Cryoglobulinemia ; diagnosis ; etiology ; Diabetes Mellitus, Type 2 ; diagnosis ; etiology ; Fatigue ; diagnosis ; etiology ; Female ; Hepatitis C, Chronic ; complications ; physiopathology ; Humans ; Lymphatic Diseases ; diagnosis ; etiology ; Male ; Middle Aged ; Thrombocytopenia ; diagnosis ; etiology ; Young Adult

We investigated the clinical features of nephrotic syndrome in Korea according to sex, age and pathologic diagnosis under the classification system supported by WHO. Adult nephrotic patients who underwent renal biopsy from 1979 to 1993 at the Department of Internal Medicine, Seoul National University Hospital, with the exception of diabetic nephropathy and multiple myeloma, were analyzed in terms of their clinical manifestations, laboratory data, and pathologic features. 1) The number of our total nephrotic patients was 956 and their mean age was 32.6. Male to female ratio was 1.96:1. 2) Among total nephrotic syndrome patients, there were 736 (77%) primary NS and 220 (23%) secondary NS patients. 3) The pathologic diagnoses of the primary NS were minimal change nephrotic syndrome (41.6%), membraneous nephropathy (20.9%), focal glomerulosclerosis (17.3%), IgA nephropathy (9%), membranoproliferative GN (4.5%), sclerosing GN (2.4%), and mesangial proliferative GN (2.2%). 4) Among primary NS patients with ages between 15 and 40, the percentage of minimal change nephrotic syndrome was highest(47.7%), while among those above 40 of age, membraneous nephropathy was most common(42.1%). 5) The pathologic diagnoses of the secondary NS were hepatitis B virus associated GN (46.8%), lupus nephritis (39.5%), amyloidosis (5.9%), post-infectious GN (2.7%), Henoch-Schoenlein nephritis (2.7%). We had one each case of thrombotic thrombocytopenic purpura, Fabry's disease, mixed essential cryoglobulinemia, light chain disease, and Alport's syndrome. In conclusion, minimal change nephrotic syndrome was the most common disease among the primary NS and hepatitis B virus associated GN was the most common among the secondary NS.
Adult* ; Amyloidosis ; Biopsy ; Classification ; Cryoglobulinemia ; Diabetic Nephropathies ; Diagnosis ; Fabry Disease ; Female ; Glomerulonephritis, IGA ; Glomerulosclerosis, Focal Segmental ; Hepatitis B virus ; Humans ; Internal Medicine ; Korea ; Lupus Nephritis ; Male ; Multiple Myeloma ; Nephritis ; Nephritis, Hereditary ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Purpura, Thrombotic Thrombocytopenic ; Seoul

Cryoglobulinemia is the presence of globulins in the serum that precipitate on exposure to cold temperatures (cryoglobulins). Pulmonary complications of cryoglobulinemia include interstial infiltration, impaired gas exchange, small airway disease and pleurisy. Only one other acute respiratory distress syndrome(ARDS) case has been described in patients with cryoglobulinemia. A 55-years old man was admitted with dyspnea. He had been diagnosed as being a hepatitis B virus antigen carrier 15 years age. On the first admission, chest radiography showed a bilateral pleural effusion and a patchy infiltration on both lungs. On protein-and immuno-electrophoresis, cryoglobulinemia was confirmed. The patient was treated with corticosteroid and plasmapheresis. Forty-five days after the diagnosis, the patient complained of progressive dyspnea and showed a diffuse bilateral pulmonary infiltration on chest radiography. Despite intensive care with mechanical ventilation, the patient died as consequence of hypoxemia and multiple systemic organ failure. On a pathologic examination of the postmortem lung biopsy, multiple necrotizing vasculitis and increased infiltration of the lymphocytes and monocytes were observed. In conclusion, ARDS developed as a result of pulmonary hemorrhage due to cryoglobulinemia-associated vasculitis.
Anoxia ; Biopsy ; Cold Temperature ; Cryoglobulinemia ; Diagnosis ; Dyspnea ; Globulins ; Hemorrhage ; Hepatitis B virus ; Humans ; Critical Care ; Lung ; Lymphocytes ; Monocytes ; Plasmapheresis ; Pleural Effusion ; Pleurisy ; Radiography ; Respiration, Artificial ; Respiratory Distress Syndrome, Adult* ; Thorax ; Vasculitis

BACKGROUND: Recently, in dermatologic area, the number of skin biopsy for diagnosis and treatment has been increased and the dermatosurgery which should give patients satisfaction developed widely. As the frequency of direct contact with patients increases, we must recognize and prevent infectious diseases such as hepatitis B and C. Also, because drugs which may affect function of liver were used in many medical clinics and shops, we have more chance of evaluating liver function before the treatment. OBJECTIVE: We investigated the prevalence and associated diseases of the patients with elevated transaminase (AST,ALT), positive of hepatitis B surface antigen and anti-HCV Ab. And we tried to study the causes of elevated transaminase. METHOD: All the 1704 outpatients who visited the dermatologic clinic of Pusan National University Hospital from March 1996 to February 1999 were enrolled. AST and ALT level were assessed by kinetic method and elevated group comprised the subjects with AST and ALT levels exceeding the upper limit of normal for our laboratories (AST >40IU/L or ALT >37IU/L). HBsAg and anti-HCV Ab were tested by enzymeimmunoassay(EIA) and medical records were reviewed. RESULTS: The prevalence of elevated transaminase was 13.6% and the peak age was the sixth decade. Most common associated disease categories were drug druption, eruption & urticaria. The prevalence of positive hepatitis B surface antigen was 9.7% and the peak age was the fourth decade (42.4%). Associated diseases of antigen positive patients were lichen planus, urticaria, cryoglobulinemia, etc. The prevalence of positive anti-HCV Ab was 3.4% and the sixth decade was the peak incidence of age distribution. The diseases such as urticaria, erythema nodosum, psoriasis, alopecia, and so on were investigated in the patients. Common suspected causes of elevated transaminase(AST, ALT) were hepatotoxic drug and viral hepatitis. Conclusion: Increased hepatic parenchymal enzyme was investigated in a high percentage of studied patients. Also, the prevalence of HBV and HCV infection in dermatologic patients were relatively higher than that of reported normal Korean people. Therefore dermatologists should recognize these facts and be careful in dermatologic procedure.
Age Distribution ; Alopecia ; Biomarkers* ; Biopsy ; Busan ; Communicable Diseases ; Cryoglobulinemia ; Diagnosis ; Erythema Nodosum ; Hepatitis B Surface Antigens ; Hepatitis B* ; Hepatitis* ; Humans ; Incidence ; Lichen Planus ; Liver ; Medical Records ; Outpatients ; Prevalence* ; Psoriasis ; Skin ; Urticaria

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.
Antigens, CD5/*metabolism ; Antineoplastic Agents/therapeutic use ; B-Lymphocytes/immunology/metabolism ; Bone Marrow/pathology ; Cryoglobulinemia/diagnosis ; Cyclophosphamide/therapeutic use ; Diagnosis, Differential ; Doxorubicin/therapeutic use ; Drug Therapy, Combination ; Female ; Glomerulonephritis/*diagnosis/pathology ; Humans ; Kidney/pathology ; Middle Aged ; Paraproteinemias/diagnosis ; Prednisolone/therapeutic use ; Vincristine/therapeutic use ; Waldenstrom Macroglobulinemia/*diagnosis/drug therapy/pathology