[Survey of one hundred patients with chronic granulocytic leukemia from the view of recent developments]. / Száz krónikus granulocitás leukémiás beteg vizsgálata az utóbbi évek haladásának szemszögéböl.

Data of 100 selected CGL patients were considered. There were 50 male and 50 female patients with an average 38.1 and 41.5 years of age, respectively. Seventy nine patients were in stable, chronic and 21 patients were in accelerated phase. Patients first admitted in blastic phase were excluded. Twenty two patients were subjected to bone marrow transplantation. Characteristically low levels of neutrophil alkaline phosphatase were absent in about 10 to 25% of early cases. It was considered that the level of positive cells could eventually point to the extent of normal population. In the group of the accelerated patients the neutrophil alkaline phosphatase scores were regularly high and the serum cholesterol values lower than those among stable-phase patients. The role of G-CSF (granulocyte colony stimulating factor) was considered. Secondary myelofibrosis was frequently associated with low serum cholesterol. Average survival time was 41 month among non-transplanted and 63+ months among identically-transplanted patients. In accordance with the literature, authors point out that in the presence of any factor not permitting transplantation, prolonged high-dose interferon-therapy could be the first choice, because unlike chemotherapeutic agents used till now, it prolongs survival. Apart from this autotransplantation with marrow or with circulating blood derived stem cells should be considered.

Permanent large granular lymphocytosis in the blood of splenectomized individuals without concomitant increase of in vitro natural killer cell cytotoxicity.

Increased numbers of circulating lymphocytes and large granular lymphocytes (LGL) were observed in 115 individuals splenectomized for haematological disease (74 cases) or for trauma (41 cases). LGL lymphocytosis was present in 78.4% of haematologically indicated and in 85.4% of traumatic splenectomies. A 70.5% of these values was above the 97.5 percentile upper tolerance limit of healthy controls (200 cases). In addition, 175 haematological controls were investigated. Forty per cent or more of circulating lymphocytes exhibited LGL morphology in nearly half of repeatedly investigated splenectomized persons. The increase in LGL is not attributable to lymphocytosis. It becomes apparent, and persists after the first postoperative week, irrespective of the cause of surgery. In spite of the two-fold increase in LGL concentration in the blood, in vitro natural killer (NK) and antibody dependent cellular cytotoxic (ADCC) activities did not increase in the investigated 48 (NK) and 31 (ADCC) splenectomized persons, as compared with the appropriate healthy or haematological controls.

Bone marrow transplantation in accelerated chronic granulocytic leukaemia using dibromomannitol-preconditioning instead of total-body irradiation.

In a preliminary study on five patients with accelerated CGL, transplantation of allogeneic matched bone marrow was shown to be feasible without whole-body irradiation. Animal experiments and studies with cells cultured in vitro suggest that the cytocastic drug used to kill leukaemic clones (Myelobromol-Chinoin) does not injure haemopoietic stroma. The administration of this protocol is cheap and easy. Our preconditioning does not, in itself, eradicate the malignant CGL clone immediately: 15-20% of marrow mitoses were Ph1+ one month after transplantation. For this reason, additional cytostatic therapy was given in the course of the 3rd to 6th post-transplant months. No Ph1+ cells were observed from the fourth post-transplant month onwards. Very few severe acute complications were seen and two out of three matched transplanted patients are disease-free 27 + and 13 + months later. On the basis of the developing normal spleen architecture and the changing pattern of circulating NAP score values, particularly the months-long persistence of distinctly low scores, and then the delayed emergence of normal levels, we put forward a hypothesis, emphasizing the role of environmental factors, including the formation of a normal haemopoietic stroma in the successfully transplanted CGL patient.