Graft-versus-host disease

The development of nuclear arms at the end of World War II was a major stimulus for research into radiation damage to the hematopoietic system and into the treatment of such damage through bone marrow transplantation [1]. A large body of research with mice, dogs, and monkeys established a “bone marrow syndrome” with death through the complications of pancytopenia after a total body dose of 5–7 Gy (500–700 rad). Such animals could be salvaged by the infusion of syngeneic bone marrow [2] or allogeneic bone marrow [3,4]. These studies were trailblazers for bone marrow transplantation as therapeutic modality in man.

Graft-Versus-Host Disease

Graft-versus-leukemia effects of transplantation and donor lymphocytes

Background Prophylactic platelet transfusions are usually administered to patients receiving myelotoxic chemotherapy when their platelet count falls below 20,000 per cubic millimeter. Some observations suggest that lower platelet counts can be appropriate in patients in stable condition, but the safety of lower thresholds is uncertain. Methods We evaluated 255 adolescents and adults (age, 16 to 70 years) with newly diagnosed acute myeloid leukemia (but not acute promyelocytic leukemia), who were treated in 21 centers. One hundred thirty-five patients were randomly assigned to receive a transfusion when their platelet count fell below 10,000 per cubic millimeter (or 10,000 to 20,000 per cubic millimeter in those with a temperature above 38°C, with active bleeding, or a need for invasive procedures), and 120 patients were assigned to receive a transfusion when their platelet count was less than 20,000 per cubic millimeter. Results Patients in the group with a threshold of 10,000 platelets per cubic millimet...

https://www.nejm.org/doi/pdf/10.1056/NEJM199712253372602

The threshold for prophylactic platelet transfusions in adults with acute myeloid leukemia. Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto

Plastic anemia, which is pancytopenia with a fatty or “empty” bone marrow, is remarkable for the simplicity of its pathologic picture and the direct derivation of its clinical manifestations.1 Although it is not a common disease, the drama of an individual case and the larger consequences of its associations give it considerable interest. That aplastic anemia is perhaps the most dreaded idiosyncratic complication of drug treatment has serious and often expensive consequences for drug development, for risk assessment, for approval by regulatory agencies, and in legal actions. Aplastic anemia, first described by Paul Ehrlich in 1888 from an autopsy of . . .

The pathophysiology of acquired aplastic anemia.

PORPHYRIA cutanea tarda, which is the most common and readily treated form of porphyria in humans, results from a deficiency of uroporphyrinogen decarboxylase in the liver.1 2 3 It is characterized by cutaneous photosensitivity. The disease is usually classified as sporadic if uroporphyrinogen decarboxylase is deficient only in the liver, and as familial (autosomal dominant) if the enzyme is also deficient in nonhepatic tissues such as erythrocytes.3 4 5 There is also a familial form in which only the hepatic enzyme is deficient.6 Increased iron content in the liver is a major contributing factor in both sporadic and familial porphyria cutanea tarda,7 as are . . .

Shung Man K. Lee

Papers

Erythropoietin for the treatment of porphyria cutanea tarda in a patient on long-term hemodialysis.