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Biomedical subjects

A Fam

Publications and source records attributed to A Fam.

16 recordsLinked to original sources
PubMed2002

Malignant evolution of Schnitzler's syndrome--chronic urticaria and IgM monoclonal gammopathy: report of a new case and review of the literature.

Schnitzler's syndrome, initially described in 1974 is an uncommon condition defined by chronic urticaria and monoclonal IgM gammopathy. Additional features include fever of unknown origin, elevated ESR, bone pain and frequently a benign clinical course. We conducted a literature search of Medline, EMBASE and Cancerlit and found 56 cases of Schnitzler's syndrome reported to date. The absence of lymphoproliferative disease in this condition is typical, but nine patients have progressed to develop lymphoplasmacytic neoplasias, particularly Waldenstrom's macroglobulinemia (WM). Malignant evolution of Schnitzler's syndrome is a rare complication, but emphasizes the importance of long term follow-up and the need for these patients to undergo periodic assessment of the bone marrow and lymph nodes. Treatment of this condition is difficult, with varying response to corticosteroids and largely unsuccessful results with standard chemotherapy used for WM. We describe a case of Schnitzler's syndrome in a 50-year old man with lymphocytic aggregates in the bone marrow after 9 years of chronic urticaria, fever, arthralgias and bone pain. We review the clinical features and treatment, with emphasis on the hematologic aspects of this unusual condition.

Bone Marrow↗
PubMed1997

Raynaud's phenomenon and digital gangrene as a consequence of treatment for Kaposi's sarcoma.

OBJECTIVE: To analyze the clinical features and to identify factors associated with the development of Raynaud's phenomenon (RP) in patients receiving chemotherapy for human immunodeficiency virus (HIV) related Kaposi's sarcoma. METHODS: A retrospective cohort study of all patients with Kaposi's sarcoma treated with chemotherapy at Toronto-Sunnybrook Regional Cancer Centre from 1987 to 1995. Patients who developed RP were compared with those who did not with respect to age, CD4 cell count, Acquired Immune Deficiency Syndrome Clinical Trials Group (ACTG) stage, smoking history, type and dose of chemotherapy, and previous treatment with interferon and radiation therapy. RESULTS: Eighty-seven patients with Kaposi's sarcoma were treated with chemotherapy between 1987 and 1995. Five developed RP, which progressed to digital gangrene. Median age, proportion of smokers, proportion defined as ACTG poor risk, median CD4 count, and history of opportunistic infections were equal in the 2 groups. All patients with RP received vinblastine followed by bleomycin. No cases of RP occurred in 27 patients treated with vinblastine alone or in 24 patients treated with bleomycin without previous vinblastine. Patients developing RP tended to have received higher cumulative doses of chemotherapy including bleomycin (p = 0.067) and previous treatment with either local radiation or interferon (p < 0.009, p < 0.001, respectively). CONCLUSION: Sequential chemotherapy with vinblastine followed by bleomycin was associated with the development of RP in patients with HIV related Kaposi's sarcoma. Bleomycin alone was not associated with RP.

Adult↗
PubMed1987

HLA-DR4 in giant cell arteritis: association with polymyalgia rheumatica syndrome.

Forty-three patients with well-documented giant cell arteritis (GCA) were studied clinically and with HLA typing. All patients were over age 40. Twenty-two of the patients had coexistent polymyalgia rheumatica (PMR). No association with class I HLA antigens was detected. When compared with HLA findings in 243 healthy controls, HLA-DR4 was increased in patients with GCA and PMR, but not in patients with GCA alone. Pooled analysis of data from 4 published papers confirmed the association of DR4 with GCA and PMR (P much less than 0.00001). Patients with PMR but negative findings of temporal artery biopsies also showed an increased frequency of HLA-DR4, but this did not reach statistical significance, probably because of the small number of subjects. It is likely that the increased frequency of DR4 in GCA patients is related to its association with PMR.

Adult↗
PubMed1973

Gonococcal arthritis: a report of six cases.

Six cases of gonococcal arthritis are described. Three presented during the initial "bacteremic stage" with polyarthralgia, fever, skin lesions and sterile synovial fluid. Two presented during the "septic joint stage" with positive synovial culture, and one presented during the "stage of residual deformity". Transient electrocardiographic changes were noted in two of the six cases. All responded to antibiotic therapy. One required additional surgical intervention.The condition is common, coinciding with the rising incidence of symptomatic and asymptomatic gonorrhea. Gonococcal infection must be considered in the differential diagnosis of migratory polyarthralgia, tenosynovitis, oligoarthritis, fever or unusual skin lesions. Criteria for diagnosis, lines of treatment and relevant literature are reviewed.

Adolescent↗