Paroxysmal night haemoglobinuria

Description:

The Paroxysmal Night Haemoglobinuria (PNH) — seldom found acquired disease caused by disturbance of an erythrocyte membrane and which is characterized by the chronic hemolitic anemia alternating or a constant haemoglobinuria and a gemosiderinuriya, the phenomena, fibrinferment and a marrow hypoplasia [Dacie, 1963, 1968]. This disease usually is for the first time diagnosed for persons in an age group of 20 — 40 years, but can meet also at elderly [Dacie, 1967; Dacie, Lewis, 1972].

Symptoms of the Paroxysmal night haemoglobinuria:

The clinical current is very variable — from soft high-quality to heavy aggressive [Dacie, 1967; Dacie, Lewis, 1972]. At a classical form hemolysis happens when the patient sleeps (a night haemoglobinuria) that can be caused by small decrease at night рН blood. However the haemoglobinuria is observed only approximately at 25% of patients, and at many not at night. In most cases the disease is shown by anemia symptoms. Hemolitic flashes can arise after an infection, a heavy exercise stress, surgical intervention, periods, hemotransfusion and introduction of iron preparations with the therapeutic purpose. Often hemolysis is followed by ostealgias and muscles, an indisposition and fever [Dacie, Lewis, 1972]. Such signs as pallor, yellowness, bronze coloring of skin and moderate splenomegaly are characteristic. Many patients complain of the complicated or painful swallowing [Rosse, 1980], often there are a spontaneous intravascular hemolysis and infections [Peytermann et al., 1972].

APG often accompanies aplastic anemia [Gardner, Blum, 1967], a preleukosis, myeloproliferative diseases and acute myeloid leukemia. Detection of a splenomegaly at the patient with aplastic anemia has to form the basis for inspection for the purpose of APG identification [Rosse, 1980].

Anemia proceeds often hard, the level of hemoglobin makes 60 g/l or below. Usually the leukopenia and thrombocytopenia meet [Rosse, 1980]. In a smear of peripheral blood the picture of a normotsitoz is, as a rule, observed, however at a long gemosiderinuriya there comes the deficit of iron which is shown signs of an anisocytosis and existence of microcytic hypochromia erythrocytes [Rosse, Gutterman, 1970]. The number of reticulocytes is raised unless insufficiency of marrow takes place. Marrow at the beginning of a disease usually гиперплазирован, however can develop further a hypoplasia and even an aplasia.

Level of an alkaline phosphatase in neutrophils is reduced, sometimes up to its total absence [Lewis, Dacie, 1965]. All signs of an intravascular hemolysis can be found, however the heavy gemosiderinuriya which leads to deficit of iron is usually observed. Besides, the chronic gemosiderinuriya causes adjournment of iron in renal tubules and dysfunction of their proximal departments [Clark et al., 1981], the Antiglobulinovy test is, as a rule, negative [Dacie, 1967].

APG has to be suspected at any patient with hemolitic anemia of not clear etiology with deficit of iron, the combined deficit of iron and folic acid, a pancytopenia, a splenomegaly and an incidental thrombogenesis. For the purpose of diagnosis use Ham's test [Ham, 1939] and the saccharose test [Hartmann et al., 1970]. These tests serve for definition of resistance of erythrocytes to small doses of a complement.

Reasons of the Paroxysmal night haemoglobinuria:

It is supposed that APG arises owing to proliferation of a defective clone of stem cells of marrow; such clone gives rise to at least three populations of the erythrocytes differing on sensitivity to the activated complement components [Rosse, 1972, 1973, 1980]. Hypersensitivity to a complement is most inherent in the young circulating erythrocytes [Hinz et al., 1956].

Treatment of the Paroxysmal night haemoglobinuria:

Symptomatic treatment as specific therapy does not exist. If there is a need for hemotransfusion, then it is necessary to use the erythrocytes washed in isotonic solution of sodium chloride [Dacie, 1948], or it is even better the frozen erythrocytes which before introduction defreeze and wash from glycerin [Gockerman, Brouillard, 1977]; such precautions are necessary because fresh donor plasma can strengthen hemolysis. Iron preparations are usually transferred well, however they can sometimes accelerate hemolysis [Hartmann et al., 1966]. The iron preparations appointed after hemotransfusion suppress an erythrogenesis [Hartmann, Kolhouse, 1972]. It was reported that at some patients corticosteroids in high doses gave good effect [Firken et al., 1968]; use of androgens [Rosse, 1980] can appear useful. Anticoagulants are shown after an operative measure, however is long them it is not necessary to enter [Hartmann, Kolhouse, 1972]. There is a number of messages on sudden development of hemolysis after administration of heparin [Crosby, 1953]. Efficiency of a splenectomy is not established, and an operation is badly undergone by patients.