Leptomeningeal myelomatosis presenting with mental status changes and other neurologic findings.

BACKGROUND: Leptomeningeal myelomatosis is a rare complication of multiple myeloma. METHODS: The authors identified and studied three patients with leptomeningeal myelomatosis and reviewed previous case reports of this condition. RESULTS: The patients described here had intermittent abnormalities in mental status or cranial nerve and brain stem abnormalities. Two of the patients responded dramatically, though transiently, to treatment. In one patient, the clinical findings correlated with lesions visualized by gadolinium-enhanced magnetic resonance imaging. CONCLUSIONS: These patients are typical of those reported previously. Patients with leptomeningeal myelomatosis often have a good response to treatment initially, but long-term survival is rare.

Plasmacytoid T-zone cell proliferation in a patient with chronic myelomonocytic leukemia. Histologic and immunohistologic characterization.

The term "plasmacytoid T-zone cells" has been used to describe distinctive cells that occur in clusters in the paracortex of some reactive lymph nodes. Recently, tumorous proliferations of these cells have been described in several patients with myelomonocytic leukemias. Neither the nature of these cells nor their relationship to myeloid leukemia has been conclusively established. We report the case of a 64-year-old woman with chronic myelomonocytic leukemia who developed lymphadenopathy that proved to be due to tumorous accumulation of plasmacytoid T-zone cells in the interfollicular regions of the lymph nodes. She underwent splenectomy because of symptomatic splenomegaly; the resected spleen also contained aggregates of plasmacytoid T-zone cells, in addition to extramedullary hematopoiesis. On treatment with busulphan and prednisone, the lymphadenopathy resolved and did not recur. The patient died 7 years later with blast transformation of her myelomonocytic leukemia and no recurrence of lymphadenopathy. The aggregates of plasmacytoid T-zone cells were architecturally and cytologically distinct from the leukemic infiltrates of myeloid cells in the spleen, and there was no evidence of differentiation of these cells into myeloid or monocytic cells. A panel of monoclonal antibodies on paraffin sections revealed no lineage-specific T- or B-cell markers (UCHL1-, L26-), and the plasmacytoid cells were positive for CD68 (KP1) and L60 (CD43), as well as faintly positive for 4KB5 (CD45RA) and MB1 (CD45R). They did not stain with antibodies to myeloid lineage antigens CD15, lysozyme, or myeloperoxidase. The combination of clinical, morphologic, and immunologic features of plasmacytoid T-zone cells in this case suggests that these cells may be of monocytic lineage but are not direct precursors of mature monocytic or granulocytic cells, and may not be part of the neoplastic clone in patients with myelomonocytic leukemia.

Sulfasalazine-induced agranulocytosis.

We report a patient with ulcerative colitis in whom agranulocytosis was diagnosed 5 wk after the initiation of sulfasalazine therapy. Concomitant features included fever and skin rash, and bone marrow examination revealed severe myeloid hypoplasia. Antineutrophil antibodies were absent from the serum. Recovery began during the 2nd wk and was characterized by a leukemoid reaction with thrombocytosis. Subsequently, the patient received 5-aminosalicylate enemas without adverse effects. Agranulocytosis, a rare effect of sulfasalazine, appears to occur almost invariably during the first 2 months of therapy, and prompt evaluation is therefore required in patients with unexplained fever or other nonspecific illness during this period.

Disseminated intravascular coagulation with the peritoneovenous shunt.

Coagulation data were collected before and after peritoneovenous shunting for intractable ascites in 19 shunting procedures. After insertion of the shunts, changes consistent with disseminated intravascular coagulation developed in all cases in which good flow of ascitic fluid was obtained. In cases with temporary shunt function, the coagulation variables suggestive of disseminated intravascular coagulation returned toward normal when the flow of ascitic fluid ceased. A fall in the level of fibrinogen degradation products indicated that the shunt had clotted. Bleeding attributable to disseminated intravascular coagulation alone was uncommon. Clotting of the shunts was frequent. The use of heparin improved some of the coagulation variables but did not prevent shunt clotting or clinical bleeding. We conclude that the peritoneovenous shunt induces a moderate disseminated intravascular coagulation and that measurement of fibrinogen degradation products is useful in assessing shunt function.

Corpus luteum hemorrhages complicating chronic anticoagulation.

A 29-year-old patient with prosthetic replacement of two cardiac valves who was receiving chronic anticoagulation therapy presented with sequential corpus luteum hemorrhages. The problems of reproductive system hemorrhage from chronic anticoagulation therapy are discussed, as well as the specific problemsof women of reproductive age with heart valve replacements. Issues and therapy are considered.