The role of spleen in the pathogeny of aplastic anemia related to increased number of CD3+ CD8+ FcR+ cells.

The role of spleen in the pathogeny of aplastic anemia (A.A.) related to excessive suppression, and the value of splenectomy in the treatment of this disorder is still debated and unclear. In an attempt to find out why some patients respond to surgery and others do not, an immunologic study was carried out in 16 patients with aplastic anemia. Lymphocytes surface markers CD3, CD4, CD8, HLA-DR, Fc receptors (FcR) and CD4/CD8 ratio were determined before and after splenectomy in the patients' peripheral blood, and in the spleen. In addition, the number of granulo-monocytic colony forming cells (GM-CFC) before and after splenectomy was estimated. Nine of the cases showed increased CD3+ CD8+ FcR+ cells, reversed CD4/CD8 ratios (both, in peripheral blood and in spleen), and a low number of GM-CFC. In all these cases, splenectomy induced an improvement of the clinical, hematological, and immunological parameters, thus suggesting that spleen represents an important "reservoir" for CD3+ CD8+ FcR+ cells, which seem to exert a suppressor effect on the hematopoietic progenitors. In splenectomized patients who did not respond to surgery, the pathogenic mechanism was probably related to defective help (3 cases with low values of CD4+ cells), to defective suppression (2 cases with decreased number of CD8+ cells), to a stem cell defect or a deficiency in the stem cell microenvironment (2 cases with normal helper/suppressor ratio). These observations support the conclusion that splenectomy is indicated and may be successful only when the phenotypic markers show an increased number of CD3+ CD8+ FcR+ cells.

Investigations of the presence of serum antibodies to Epstein-Barr virus in patients with various tumors.

Serum samples from 150 patients with various malignant or benign tumors and from 20 clinically healthy subjects were tested by indirect immunofluorescence (IF) reaction for the presence of specific antibodies to Epstein-Barr-virus-determined viral capsid antigen (EB VCA). The IF test-carried out in multitest-slide EB3 and Raji cell preparations-revealed antibodies to EB VCA in 102 of the patients with tumors and in 4 of the healthy persons.

Polycythemic blood perfusions in severe aplastic anemia.

The course of two cases of severe aplastic anemia (AA) was followed up in two young male patients who, besides the conventional therapy, received perfusions with blood obtained from patients with polycythemia vera (PV). Case 1 received 4 units of PV blood from a unique donor. During the following week an obvious clinical relief, as well as increase of the peripheral blood values were recorded. Subsequently, the bone marrow was progressively repopulated with normal precursors and the peripheral blood picture reverted to normal. In case 2, 9 units of PV blood, obtained from two donors, were administered. During the next week a transient clinical and hematological improvement was obtained, but later on the progressive granulocytopenia and the infections led to exitus through a toxic-septic shock. It is assumed that PV blood may be a good replacing and a possible stimulatory therapeutic material, useful to overcome the early critical stage of severe AA.

Congenital dyserythropoietic anemia type II. Radioautographic, ultrastructural and cytogenetical study in two familial cases.

The case of two sisters is reported in whom morphological (multinucleated erythroblasts in the bone marrow) and serological (positive Ham test, negative sucrose and water-sugar tests) characteristics of a congenital dyserythropoietic anemia type II were found. Radioautographic, cytogenetic and electron microscope studies confirmed the presence of an inefficient erythropoiesis and of a membrane defect, common in this disease. The finding of a nil LAP score has never been reported so far.