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A 64-year-old woman with a 20-year history of polycythemia vera and a 2-month history of postpolycythemic myeloid metaplasia ( PPMM ) became comatose because of bilateral subdural hematomas. Biopsy of the dura mater at operation demonstrated extramedullary hematopoiesis ( EMH ). The subdural hemorrhage was secondary to dural invasion by hematopoietic tissue and vascular dilation with a predisposing element of thrombocytopenia. This is the second case of central nervous system (CNS) EMH in a case of polycythemia vera. Only 8 cases of symptomatic CNS EMH and 16 cases overall have been reported, and these were primarily due to agnogenic myeloid metaplasia. Symptoms were secondary to the mass effect of dural tumor-like hematopoietic tissue or to an elevated cerebrospinal fluid protein content. The survival after a diagnosis of PPMM with CNS EMH has been less than 6 months in all cases. CNS EMH is an unusual and a potentially critical complication of polycythemia vera that should be considered in the evaluation of the patient with polycythemia vera and myeloid metaplasia.

Fibrillary renal deposits and nephritis. The authors have studied 8 patients whose glomeruli contain abundant fibrils in their mesangial matrix and basement membranes. Although the location of these fibrils is very similar to that of amyloid, they are about twice the size of amyloid fibrils, averaging 20 nm in width, and fail to react as amyloid does with special stains. Immunofluorescence-microscopic studies are usually positive with antiserums to IgG, often IgM, and in some cases IgA, and also kappa and lambda light chains, C3, and C4. The fibrils are associated with diffuse mesangial widening and increased mesangial matrix strands. Although peripheral glomerular capillary walls appear to be spared initially, their eventual involvement leads to glomerular capillary collapse and glomerular obsolescence. Crescent formation occurred in 5 cases, focally in 3 and diffusely in 2. Tubular basement membrane involvement was seen in 1 case. These patients exhibit hematuria, and proteinuria, and often hypertension and renal insufficiency. Proteinuria was in the nephrotic range in 3 patients in whom involvement of glomerular capillary basement membranes was extensive. Unless electron microscopy is applied to renal biopsies, these cases may be considered to represent mesangiocapillary or rapidly progressive glomerulonephritis, or amyloidosis. The nature of these fibrils is as yet not determined. It is likely that they have been called "atypical amyloidosis" in the past.

We report a case of a vesicouterine fistula with menouria (vesical menstruation) secondary to an intrauterine contraceptive device. Of the 23 cases of menouria reported previously 21 occurred after cesarean section, 1 was secondary to a traumatic forceps delivery and 1 was owing to infection. In our case the fistula did not close after removal of the perforated intrauterine contraceptive device and 2 months of catheter drainage. Closure was achieved by excision of the fistula and hysterectomy.

Metastatic pulmonary calcification, a well-known complication in patients with chronic disease, has been demonstrated postmortem in patients with a negative chest X-ray. Recently, scintigrams with bone-seeking radionuclides have been used to detect such subclinical pulmonary calcium deposits. We describe 23 patients on maintenance hemodialysis with no evidence of pulmonary calcification on chest X-ray who were prospectively studied by lung scanning with a bone-seeking radionuclide and pulmonary function testing. Of the 23 patients, 14 (61%) had a positive technetium-99m diphosphonate (99mTc-DP) scan (group 1). These patients were on dialysis 38 +/- 5 months compared with 12 +/- 4 months in 9 patients with a negative scan (group 2) (P less than 0.01). Age, sex, blood pressure, hematocrit, serum calcium, phosphorous, bicarbonate, magnesium, and calcium X phosphorus product, as well as parathyroid hormone level did not differ between the two groups. Of 10 group-1 patients tested, 7 had abnormal pulmonary diffusion capacity compared with non in 5 group-2 patients tested (P = 0.014). Histologic examination of the lung in 1 group-1 patients who expired revealed calcification (amorphous on X-ray diffraction), whereas none was found in 1 group-2 patients autopsied. These observations suggest that in patients on maintenance hemodialysis, pulmonary scanning with 99mTc-DP is a sensitive method for detecting pulmonary metastatic calcification, which may be associated with an abnormality in pulmonary diffusion capacity.

In this report, we describe the case of a boy who developed the nephrotic syndrome at one and one-half years of age, remained steroid-responsive for nine years, but then became steroid-resistant, associated with the development of progressive glomerular sclerosis and renal insufficiency. We suggest that this evolution is evidence in favor of the view that minimal change disease and focal glomerulosclerosis have a common pathogenesis.

A patient with Hodgkin's disease associated with low glucocerebrosidase levels in the peripheral leukocytes, and Gaucher's cells in the bone marrow and lymph nodes, is described. After MOPP therapy, complete remission of Hodgkin's disease was accompanied by normalization of the glucocerebrosidase level and disappearance of Gaucher's cells. This observation appears unique when compared with the four cases of combined Hodgkin's and Gaucher's disease reported in earlier literature in which Gaucher's disease remained unchanged after chemotherapy. We conclude that our patient had Hodgkin's disease and acquired Gaucher's cells with diminished glucocerebrosidase levels, rather than a combination of Hodgkin's disease and Gaucher's disease.

Two HLA identical siblings developed focal glomerulosclerosis at about 15 years of age. To our knowledge, this is a unique case of the familial nephrotic syndrome because this is both the latest onset ever reported and the first description of HLA identity. We suggest that the pathogenesis of renal disease in these siblings involved an interaction of a shared genetic susceptibility and a common environment.

Primary localized amyloidosis of the ureter is very rare, and only 22 cases have been reported in the world literature. We report the twenty-third case along with a review of the relevant literature. Due to its radiologic resemblance to malignancy, many cases were treated by nephroureterectomy in the past. The case being reported here was successfully treated by local excision and ureteroneocystostomy.