Carbon monoxide production associated with ineffective erythropoiesis.

The rate of endogenous carbon monoxide production ( Vco), determined by the closed rebreathing system technique, was elevated above the normal range in four of five patients studied with ineffective erythropoiesis (four patients with primary refractory anemia, one with thalassemia). The mean molar ratio of Vco to Vheme (rate of circulating heme catabolism, determined from (51)Cr red cell survival curves) was 3.0 +/- 0.6 (SE), indicating that most of the CO originated from sources other than circulating erythrocyte hemoglobin, in contrast to previous findings in patients with hemolytic anemia, where Vco paralleled Vheme closely.After administration of glycine-2-(14)C to these patients, endogenous CO was isolated by washout of body CO stores at high pO(2) or by reacting peripheral venous blood samples with ferricyanide. The CO was then oxidized to CO(2) by palladium chloride and trapped for counting in a liquid scintillation spectrometer. "Early labeled" peaks of (14)CO were demonstrated which paralleled "early labeled" peaks of stercobilin and preceded maximal labeling of circulating heme. Production of "early labeled" (14)CO in patients with ineffective erythropoiesis was greatly increased, up to 14 times that found in a normal subject. The increased Vco and "early (14)CO" production shown by these patients are presumably related mainly to heme catabolism in the marrow. The possibility exists that hepatic heme and porphyrin compounds may also contribute significantly to Vco, as suggested by the finding of a high Vco in an additional patient with porphyria cutanea tarda.

Giant kidneys in Waldenström's macroglobulinemia.

A patient with Waldenström's macroglubulinemia had the unique feature of having one kidney infiltrated with malignant cells to such an extent as to produce a large palpable abdominal mass. Other unusual aspects of this case include the destruction of renal tubules by the invading cells that resulted in interstitial fibrosis and was probably responsible for the appearance of whole IgM in the urine.

A hypereosinophilic syndrome mimicking rheumatoid arthritis.

A 58 year old white man presented with an articular syndrome that appeared clinically to be rheumatoid arthritis with nodules. Lack of response to therapy, and laboratory and pathologic data failed to confirm the diagnostic impression. The patient's course over the ensuing seven years was compatible with the hypereosinophilic syndrome. The articular involvement represented soft tissue and synovial fibrinoid degeneration with eosinophilic infiltration. This is the first reported case of articular involvement in the hypereosinophilic syndrome and adds it to the list of processes that can cause hand deformity resembling rheumatoid arthritis.

Renal transplantation for end-stage myeloma kidney: report of a patient with long-term survival.

A patient had prolonged survival after renal transplantation was performed for myeloma kidney. Continued function of the allograft without evidence of recurrent myelomatous involvement suggests that transplantation be considered for patients with multiple myeloma in whom renal failure constitutes the major problem.

Testicular plasmacytoma.

The case of a sixty-five-year-old man with multiple myeloma and a testicular plasmacytoma is described. This represents the thirty fourth reported case of testicular plasmacytoma and the first in which immunoperoxidase histochemistry has been used to demonstrate that the testicular plasma cells contain immunoglobulin of the same isotype as the patient's paraprotein. The clinical and morphologic features of previously reported testicular plasmacytoma are reviewed.

High dose dapsone toxicity.

A description of the clinical and biochemical course is given for a patient who developed haemolysis and methaemoglobinaemia following dapsone therapy in high dosage for 2 weeks.

Hypersplenism in advanced breast cancer: report of a patient treated with splenectomy.

A patient with widespread metastatic breast cancer had thrombocytopenia and severe anemia due to splenic hyperfunction, confirmed by chromium51-labeled red cell survival and sequestration studies. Marked splenic enlargement was produced by metastatic tumor. After she failed to respond to steroids, her hematologic status was improved by splenectomy, and has been stable for 16 months. Hypersplenism may be suspected as a cause of severe hemolytic anemia in advanced carcinoma. If the patient's general status is otherwise compatible with long comfortable survival, appropriate diagnostic studies and consideration of splenectomy are warranted.

Cytapheresis in a patient with Sezary syndrome.

A patient with Sezary syndrome underwent cytapheresis on the IBM 2997 cell separator. Four procedures were performed over a 12-day period. A total of 8.3 x 10(10) Sezary cells were removed. The patient's skin lesions and lymphadenopathy regressed. Transient thrombocytopenia occurred, requiring platelet transfusion. Although a significant number of platelets were removed by pheresis, this was not sufficient to explain the thrombocytopenia. The patient subsequently died of causes unrelated to the cytapheresis.

Clinical manifestations of intrathoracic cutaneous T-cell lymphoma.

Intrathoracic involvement with cutaneous T-cell lymphoma (CTCL) was documented in eight patients with mycosis fungoides and two patients with Sezary syndrome. The radiographic findings consisted of multiple bilateral parenchymal nodular densities (five patients), patchy areas of consolidation (two patients), diffuse reticulonodular or interstitial infiltration (two patients), and pleural effusion without underlying parenchymal disease (one patient). Dyspnea on exertion and nonproductive cough were the most frequent presenting symptoms, and physical examination of the lungs was usually normal. In one patient a partial Pancoast's syndrome developed from a pulmonary apical mass. Results of blood gas studies and pulmonary function tests indicated an alveolar-capillary block in gas diffusion. Although the antemortem diagnosis was often suggested on cytopathologic preparations or on tissue obtained by transbronchial or percutaneous needle aspiration biopsy, a definitive diagnosis of CTCL usually required an open-lung biopsy. The response of pulmonary infiltrates to various systemic chemotherapeutic agents was variable, and the mean survival after initiation of drug therapy was 9.5 months. The findings suggest that combined modality therapy should be considered for patients with extracutaneous CTCL.