Libman-Sacks endocarditis in the antiphospholipid syndrome: immunopathologic findings in deformed heart valves.

OBJECTIVE: To examine the potential immunologic mechanism and involvement of antiphospholipid antibodies in the pathogenesis of heart valve lesions in patients with the antiphospholipid syndrome (APS). METHODS: Immunoperoxidase and immunofluorescence staining methods were used to evaluate 13 heart valve specimens derived from eight patients with the APS, either primary or secondary to systemic lupus erythematosus. Primary antibodies to human immunoglobulins, complement components, serum albumin and a monoclonal anti-idiotypic antibody to human anticardiolipin antibodies (aCL) were employed. Various tissue specimens from a patient with the APS as well as deformed and normal valves from subjects without the APS were used as controls. RESULTS: Linear subendothelial deposition consisting of immunoglobulins with complement components but not of a non-specific serum protein was found in deformed valves from patients with the APS. None of the control valves or tissues disclosed similar deposition. The same pattern and location of staining was obtained by the anti-idiotypic antibody to aCL. A significant amount of IgG immunoglobulins that bound to cardiolipin was eluted from a valve of a patient with secondary APS. CONCLUSION: Deposits of immunoglobulins including aCL, and of complement components, are common in affected valves of patients with primary and secondary APS. Such deposits may be involved in the pathogenesis of valvular lesions.

Relapse of aggressive myeloma after complete remission in secondary acute leukemia: coincidence or consequence?

We describe a patient with multiple myeloma who developed secondary acute myelomonocytic leukemia after long-term melphalan treatment. Following two courses of low-dose cytarabine, complete remission of the A.M.L. was achieved. Shortly thereafter an aggressive relapse of the quiescent myeloma occurred with acute renal failure and massive infiltration of bone marrow with multinucleated giant plasma cells. Although it is well known that administration of melphalan to patients with multiple myeloma increases the likelihood of A.M.L., this case demonstrates that treatment of A.M.L. in a patient with multiple myeloma may perhaps influence the course of multiple myeloma.

[Relapse of multiple myeloma in extramedullary sites].

Multiple myeloma was diagnosed in a 69-year-old man with pathological hip fracture, who had increased plasma cells in the bone marrow, osteolytic lesions in bones, low level of monoclonal protein (IgAK) in serum and urine, and anemia, hypercalcemia and renal insufficiency. He was treated for 1 year with chemotherapy with good results. 6 months after cessation of treatment the disease relapsed with multiple extramedullary plasmacytomas in skin and subcutaneous areas, right eyebrow, right knee, sternum and right axilla, but repeated bone marrow examinations were without evidence of disease activity. Chemotherapy was resumed but the patient died 1.5 years after the relapse with severe jaundice due to multiple liver plasmacytomas and pelvic masses.

Recurrent peripheral arterial occlusion by leukemic cells sedimentation in acute promyelocytic leukemia.

A 10-year-old boy presenting with recurrent successive events of peripheral arterial occlusion in both legs, more prominent on the left side, was diagnosed as having acute promyelocytic leukemia. Histological examination of the occluding material showed that it was composed of leukemic blast cells. The boy underwent five operations. Each time the arterial flow was reestablished by removing cylindrical hollow white material obstructing the lumen. However, after the last operation, irreversible ischemia developed, necessitating an above-knee amputation. There was no evidence of disseminated intravascular coagulation. Three months later a right lumbar sympathectomy was performed for relief of causalgic-ischemic pains, with successful results. Preoperative angiography demonstrated irregular narrowing of the right iliac arteries and tibioperoneal trunk stenosis.

Retroperitoneal liposarcoma presenting with bilateral leg lymphedema--case report.

The case of a 54-year-old man with a large right retroperitoneal liposarcoma presenting with bilateral leg lymphedema, is reported. Total excision of the tumor resulted in marked reduction of the edema in both legs persisting for four years. Follow-up lymphographies demonstrated lymphatic drainage dysfunction on both sides; nevertheless, the edema did not recur. Based on these findings, a non-compressive hypothesis concerning the evolution of lymphedema in this case is postulated.

Malacoplakia presenting as a pseudotumor of the bladder in cadaveric renal transplantation.

Genitourinary malacoplakia in renal transplant patients is rare. We report a case of malacoplakia of the bladder, which presented as a vesical tumor.

Heterotopic pancreatic tissue in the gallbladder.

An unusual case of heterotopic pancreatic tissue found incidentally in an inflamed gallbladder is reported. The literature relating to aberrant pancreas, its origin and diverse locations, is reviewed.