Eritroblastopeniya (Krasnokletochnaya aplasia)

Description:

The partial krasnokletochny aplasia (PKKA) is a syndrome with sharp suppression of products of erythrocytes isolated by normokhromny anemia and a deep reticulocytopenia.

For the first time PKKA described Kaznelson in 1922. Further a number of cases of this disease was described, at the same time at a considerable part of patients the thymus tumor is revealed (thymoma).

In the subsequent there were descriptions of the PKKA inborn form which was shown in the first 2 years of life. Now no more than 300 sick PKKA are described and shown that it not one, and several various diseases. In one cases even at long observation of the patient it is not possible to reveal bonds of PKKA with any other diseases (PKKA idiopathic form). In other cases of PKKA it is connected with a thymus tumor; it becomes frequent this syndrome the first manifestation of any hemoblastosis. Some authors allocate the PKKA special (teenage) form with a favorable current.

The partial krasnokletochny aplasia belongs to rare diseases (about 300 such patients are described now), occurs at persons of various age, is more often at elderly people.

Eritroblastopeniya's symptoms (Krasnokletochny aplasia):

As well as at other types of anemia, the complaint of patients come down to increased fatigue, the progressing weakness, heartbeat, an asthma at insignificant exercise stresses. Pallor of integuments and visible mucous attracts attention; on a top of heart systolic noise is listened, the liver, a spleen are not increased, the hemorrhagic syndrome usually is absent.

Main hematologic displays of a disease: reduction of quantity eritro-and normoblasts in marrow punctates often at relative safety of other sprouts of a hemopoiesis and its general cellularity, deep refractory normokhromny anemia, a reticulocytopenia; the number of leukocytes, thrombocytes within norm or is lowered, the leukocytic formula normal or comes to light its shift to myelocytes to the left.

Cases meet increase in quantity eritro-and normoblasts in marrow though in connection with an inefficient eritropoee the maintenance of erythrocytes and reticulocytes in peripheral blood is reduced.

At a marrow trepanobiopsiya the hypoplasia usually does not come to light. Content of iron in bodies and blood serum is increased. Direct test of Koombs, as a rule, negative. However, use the unit-gemagglyutinatsionnoy of test allows to reveal antibodies on a surface of the erythrocytes in most cases belonging to the class IgA or to IgG or to a combination of IgA and IgG.

Rather accurate description of a clinical picture of a partial krasnokletochny aplasia as syndrome at other diseases is not available yet. Most of all data on features of its current at patients with a thymoma. Along with refractory anemia, a reticulocytopenia, an eritroblastopeniya the myasthenic syndrome (a diplopia, muscular weakness, difficulty of swallowing and chewing, twang of a voice) is noted; the syndrome of a mediastinal compression (short wind, cyanosis, hypostasis of area of a neck, breast, swelling of jugular veins) can be expressed. The X-ray analysis and a tomography of bodies of a thorax reveal blackout of a front mediastinum. Content of serumal iron is increased. The electrophoresis of proteins finds increase in fraction at - globulins, an immunoelectrophoresis — increase in the IgA and IgG level.

The inborn form of a partial krasnokletochny aplasia occurs only at children of early age (from 1 month to 1 year), is frequent at premature. Miyelogramma is characterized by an aplasia of an erythroblast sprout, in a gemogramma — anemia, a reticulocytopenia.

Eritroblastopeniya's reasons (Krasnokletochny aplasia):

The etiology of a partial krasnokletochny aplasia in most cases remains unspecified, at a number of patients developing of a disease can be connected using various medicamentous drugs of miyelodepressivny action. It is suggested about immune genesis of a disease (in plasma find the antibodies which are relating to IgG, fixed on a kernel of normoblasts and erythroblasts and having cytotoxic effect on these cells in a part of patients). Some patients at plasma have the erythropoetin inhibitor representing antibodies to erythropoetin, relating also to IgG.

Mechanisms of development of a partial krasnokletochny aplasia as the syndrome accompanying other diseases remain not clear. There are only messages on identification in plasma of the antibodies sick with a thymoma possessing strictly specific action against nuclear antigen of erythroblasts.

Eritroblastopeniya's treatment (Krasnokletochny aplasia):

The main method of treatment of a partial krasnokletochny aplasia is transfusion of eritrotsitny weight. In certain cases favorable results are achieved when using a plasma exchange, corticosteroid and anabolic hormones, immunodepressive means (Cyclophosphanum, 6 Mercaptopurinum, etc.). If the applied means are inefficient, it is necessary to resort to a splenectomy.

At an inborn form of a partial krasnokletochny aplasia systematic transfusions of eritrotsitny weight promote that children live to 8 — 15-year age then at part of them there occur spontaneous improvement and need for a hematotherapy disappears if improvement does not arise, the disease gets further the same current, as well as from adults. At development of a partial krasnokletochny aplasia against the background of a thymoma use radiation or its operational removal.