Erythromelas

Erythromelas (synonym: erythroleucus, disease Di Guglielmo) is one of the forms of acute leukemia characterized by the presence in the blood of a large number of nuclear erythrocytes - eritro - and normoblastov. The disease occurs with increasing severe anemia, fever, enlarged liver and spleen and phenomena hemorrhagic diathesis in the late stages of the disease.
Diagnosis of erythromelas put on studies of the blood and bone marrow. In blood anemia with a pronounced anisocytosis, a significant number of erythroblasts at different stages of maturation, the amount of blood can vary from 3000 to 25 000-30 000, thrombocytopenia. In the late period of the disease, the blood picture is different prevalence of blast cells. Bone marrow rich immature cells of the red line, often similar to megaloblastic. The disease usually progresses rapidly; Outlook in most cases, the adverse.
Treatment of erythromelas is the same as acute leukemias (see).

Erythromelas [from Greek. erythros red, myelos - marrow (bone)] is a systemic disease of blood by type of leukemia, with simultaneously expressed eritropoeticescoe proliferation. The greatest practical importance acute erythromelas, or syndrome Di Guglielmo.
The disease most often occurs on the type of acute leukemia (see) with a high fever, anemia and phenomena hemorrhagic diathesis; it is often stated subikterichnost of the skin caused by hemolysis of erythrocytes. In some cases, increased lymph nodes, liver and spleen. Less often the temperature is subfebrile, the disease develops gradually and is long-term.
The diagnosis is based on analysis of peripheral blood and bone marrow. In the blood was primarily expressed normohromnaja or hyperchromic anemia with a large number of normoblastov and even megaloblastic cells (malignization erythroblast), anisoles with a tendency to macrocytes, poikilocytosis of red blood cells, the presence of the Taurus Jolly and rings Kubota. Sometimes 100 of leukocytes in peripheral blood have 100-200 normoblastov and more. The number of cells or in the normal range, or increased to 20 000-30 000 1 mm3; often there is leukopenia with a shift to the left. The platelet count decreased, there is anisoles them, often lack processes, vacuolization of granulometry and Galomar.
In the study of bone marrow in typical cases, there is a marked hyperplasia due to the increase in the number of normoblastov and reticular cells in reducing the number of megakaryocytes and elements leikopoaiza. This indicates a sharp limit the ability of genocidalists to differentiate towards leiko - and thrombocytopoiesis in strengthening their ability to differentiate into erythroblast and normoblast.
Among erythroblasts is dominated by large, basophilic and the so-called megaloblastnye erythroblast [proerythroblast Rohr (R. Rohr)]. The number of genocidalists also increased (10-15%), the total number of erythroblasts and normoblastov reaches 50-60 per cent, with a predominance of immature elements, lost the ability to differentiate into the side of the Mature erythrocytes; this explains the presence of severe anaemia in these patients.
The number of elements myeloid series or normal, or there is a marked prevalence of promielozitov and plasmic order has been revealed.
If patients die at this stage of the disease, the disease is considered as a sharp erythromelalagia. If one manages to save the patient's life for a longer time, then there is a transformation of bone marrow: the observed dynamic transitions from eritropoeticescoe bone marrow to genocideallafrica.com or myeloblastic. The disease acquires the features characteristic of acute leukemia (genocidalists or myelosis), with appropriate clinical and hematological picture.
However not in all cases there is a line between the picture of the bone marrow and peripheral blood; in some cases the blood has a large number of normoblastov (erythroblasts), and in the bone marrow, there is no increase in the number of erythroblasts and normoblastov if there is a high percentage of genocidalists or myeloblasts.
The emergence of a large number of erythroblasts in the blood in these cases is explained, according to pop, hit them in the blood of the seats of extramedullary hematopoiesis. Along with sharp eritromitinom exists and subacute erythromelas, which is more long (3-5 years or more; in these cases, there is also normoblasts in the bone marrow and at the same time hypochromic anemia and leukopenia. The liver and spleen were enlarged, nerezko expressed hemorrhagic symptoms, hemolysis with the increase of bilirubin in the blood. There are mixed forms of acute and subacute eritropoaiza, in particular retroperitonealny myelosis (Di Guglielmo, I. A. Cashiers). In these cases, in the bone marrow along with eritro - and normoblasts there is an increasing number of megakaryoblastic and megakaryocytes; in peripheral blood is observed hypertronics.
A very controversial question of the existence of chronic erythromelas described Beaune (J. M. Vaughan). Most authors (I. A. Cashiers, K. A. Hasanov and others) considers this disease to the group of osteomalacia, it eritropoeticescoe option.
The disease usually develops slowly; patients concerned about the weakness, heaviness in the left side, sometimes pain in the bones and moderate diarrhoea disorders. Objective examination, in addition to the paleness of the skin and visible mucous membranes, draws attention distinct enlargement of the spleen, which is dense and painless; to a lesser extent increased and liver.
The blood test gives a moderate anemia, small leukocytosis (15 000 - 25 000) or even the normal number of white blood cells; however, in the hemogram, in addition myeloblasts, promielozitov and plasmic order has been revealed, some normoblast (15 - 30%). In the study of bone marrow find a large number of erythroblasts (normoblastov), many reticular cells and megakaryocytes. The marrow puncture gives a picture pronounced myelofibrosis with endoteliem the osteosclerosis. X-rays of the bones detects thickening of the spongy substance, the narrowing of the medullar channel. The disease is relatively favorably, and rather long-term (10-20 years and more).
The diagnosis of all forms and variants of erythromelas difficult. The differential diagnosis should be carried out with anaemia: pernicious (B12-deficiency), gipoplasticheskaya acquired hemolytic, with Cooley's anemia, tumor form systemic erythroblastosis, with erythroblastosis newborn and symptomatic when cancer metastases in bone marrow. Treatment of patients with eritromitinom is the same as with acute leukaemia, in particular she hormonal drugs (corticosteroids) and transfusions erythrocyte mass.