Tumorous phosphaturic osteomalacia. Report of a case associated with multiple hemangiomas of bone

-

sarcoidosis.

masses

Am J Med 67:

Testicular sarcoidosis

147-150.

phosphaturic osteomalacia. Report of a case asof bone. Am J Med 67: 155-159, 1979.

Phosphaturia

A 34 year old man presented with back pain and generalized muscular weakness. He was found to have hypophosphatemia, normocalcemia, markedly increased alkaline phosphatase. decreased tubular reabsorption of phosphorus, hyperphosphaturia and renal glycosuria. Skeletal roentgenograms revealed multiple osteolytic bone lesions and generalized osteopenia. Biopsy of the osteolytic lesions of the pelvis and of the skull disclosed sclerosing hemangioma of bone. His bone pain and myopathy responded to treatment with oral phosphate and vitamin D. The literature on hypophosphatemic phosphaturic osteomalacia associated with mesenchymal tumors is reviewed.

Daniels RA, Weisenfeld I: Tumorous sociated with multiple hemangiomas

Hypophosphatemia

of bone

Osteomalacia

Multiple hemangiomas

We describe a 20 year old black man who presented with shortness of breath, hilar adenopathy, monoarticular arthritis, polydipsia, polyuria and bilateral testicular masses. Transbronchial biopsy revealed noncaseating granulomas consistent with sarcoidosis. Concern over the possibility of a testicular neoplasm led to right testicular biopsy which revealed noncaseating granulomas consistent with testicular sarcoidosis. Steroid therapy resulted in clinical improvement and complete resolution of the remaining left testicular mass.

DL, Wofeldt FD: Testicular

Opal SM, Pittman 1979.

Genital sarcoidosis

Hypothalamic

Sarcoidosis

Sarcoid arthropathy

Progressive

systemic Hematologic

sclerosis

Scleroderma dyscrasia

activity

Chlorambucil Fibroblastic

factor

Systemic

vasculitis

Lymphadenopathy

sarcoma

Generalized lymphadenopathy and other manifestations of Sjbgren’s syndrome developed in a 68 year old woman with a long history of systemic vasculitis and arthralgia. An unusual immunologic feature was hypogammaglobulinemia and IgA monoclonal immunoglobulinemia with mixed IgA-IgG cryoglobulin. At autopsy, the histopathologic findings were compatible with immunoblastic sarcoma. The monoclonal IgA protein, found in serum, pleural and pericardial fluids, showed rheumatoid factor activity. lmmunocytes from the immunoblastic sarcoma were found to be the source of the monoclonal IgA protein.

and sar-

Cryoglobulinemia

lmmunoblastic

Aizawa V, Zawadzki ZA, Micolonghi TS, McDowell JW, Neiman RS: Vasculitis Sjdgren’s syndrome with IgA-IgG cryoglobulinemia terminating in immunoblastic coma. Am J Med 67: 160-166. 1979.

IgA rheumatoid

Sjogren’s syndrome

A case of acute myelofibrosis subsequent to long-term chlorambucil therapy for progressive systemic sclerosis (PSS) is reported. This is apparently the first such case to be reported in association with chlorambucil therapy as well as the first documented case of acute myelofibrosis in a patient with PSS. That altered fibroblastic activity. vascular or immunologic factors may play a role in the pathogenesis of PSS is suggested by this combination of illnesses. There exists preliminary evidence to support such a role; however, its clarification requires further investigation.

Gisser SD, Chung KB: Acute myelofibrosis in progressive systemic sclerosis. Report of a case developing during long-term chlorambucil therapy and discussion of pathogenetic factors. Am J Med 67: 151-154, 1979.

Myelofibrosis