Morphological demonstration of the typical Sézary cell in peripheral blood confirmed the diagnosis of Sézary syndrome in a 64-year-old patient with generalized erythrodermia and typical histopathological skin changes. Enzymes and immunocytological membrane characteristics of Sézary cells changed in the course of the disease after cytostatic therapy. Polychemotherapy clinically led to transitory complete regression of skin changes. Reviewing the few published investigations it is found that the morphologically sufficiently defined Sézary cells are apparently not uniform cytochemically and immunocytologically in different patients.

Autopsy↗

PubMed1980

Sézary syndrome: immunocytological and cytochemical variability of Sézary cells.

The characteristic large cells in the blood of a patient with Sézary syndrome underwent immunocytological and cytochemical changes during polychemotherapy, which caused transient regression of skin lesions. Tartrate-resistant acid phosphatase was demonstrable in a few cells only after chemotherapy; initially, only 2% T cells and 26% B cells could be demonstrated in the blood by immunocytological methods; after cytostatic therapy, 35% T cells but no B cells were detected.

Acid Phosphatase↗

PubMed1980

[Growing bone islands as differential diagnosis of osteoplastic metastases (author's transl)].

A case with two growing bone islands is reported. In one of the lesions a biopsy was performed, the other one showed a positive scintiscan. Metastases could be excluded. The most important differential diagnosis is osteoplastic metastases. The etiology of the growing bone islands is unknown.

Bone Diseases↗

PubMed1978

Role of erythropoietin in the anemia of renal insufficiency in man and in an experimental uremic rabbit model.

ESF deficiency is probably not a major contributing factor in the early stages of the anemia of renal insufficiency. Serum ESF titers are lower in advanced renal failure when compared to that of nonuremic anemic subjects suffering from equivalent anemia. With increasing renal insufficiency a relative ESF deficiency gains increasing importance as a pathogenic factor in reduced erythropoiesis. Kidneys without excretory function may still be erythropoietically effective, since a further increase in the anemia occurs after bilateral nephrectomy. However, a basal erythropoiesis is still maintained by extrarenal ESF production, which is also enhanced by hypoxia. ESF deficiency is compensated after successful renal transplantation. A decreased response of the bone marrow to ESF may be another factor contributing to the hypoproliferative state of erythropoiesis in uremia. As demonstrated in a chronic uremic rabbit model there may be a blockade of further differentiation of the erythroid precursors. The relationship of this blockade in differentiation to the inhibitor of heme synthesis is not clear.

Anemia↗

PubMed1977

[Plasma erythropoietin and hematocrit under the influence of chronic hemodialysis treatment (author's transl)].

Hematocrit and plasma ESF titers were determined at 2 to 3 weeks intervals in 21 patients with chronic renal failure prior to and during a 15-weeks' period following initiation of chronic intermittent hemodialysis. While hematocrits increased from 22 to 27%, plasma ESF titers were found unchanged between 31 and 35 mU/ml. It can be excluded therefore that the improvement of erythropoiesis following initiation of dialysis was in part due to an increase in plasma ESF titers. The increased erythropoiesis observed is probably not dependent on increased ESF production. A 30-fold ESF deficit existed in patients with renal failure prior to the initiation of hemodialysis when compared with 5 patients with aplastic anemia (hematocrit 23%, plasma ESF titer 1115 mU/ml). At one exception ESF titers up to 500 mU/ml were found in dialysed patients only in combination with anemia due to acute bleeding or iron deficiency. ESF production is appropiate to the degree of anemia in patients with proper renal function after kidney transplantation.

Adult↗

PubMed1975

[Congenital leucosis with unusual involvement of the brain(author's transl)].

A case of congenital paraleucoblast-leucosis is reported. The diagnosis rested on pronounced infiltrations of the skin and leucemic signs in blood and bone marrow. The child died on the 20th day after developing signs of bleeding and cerebral involvement. At necropsy leucotic infiltration was found in may organs, mainly in pancreas, skin and bone marrow. The most prominent findings and cause of death were diffuse hemorrhagic foci with a high proportion of leucemic cells in the brain and cerebellum.

Acute Disease↗