Chronic erythromyelosis
Chronic erythromyelosis related to leukemia, originating from a common progenitor cell myelopoiesis, represented mainly erythrokaryocytes. From acute erythremic myelosis this process is different the absence or presence of only a small percentage of erythroblasts and myeloblasts, as well as a stop at the level of differentiation erythrokaryocytes basophilic and ortohromnyh normocytes.
Clinical manifestations of chronic erythremic myelosis
Clinically, the disease manifests itself persistent norm- or hyperchromic macrocytic anemia. The level of reticulocytes in blood normal or slightly elevated (to 2 %). Along with this, there is a high normocytosis. In bone marrow hyperplasia pronounced notes of red sprout, often megaloblastnaya nature, although the level of cobalamin (vitamin B12) the serum is not reduced.
All of these changes: anemia without high reticulocytosis, Hyperplasia of red sprout in the bone marrow - are indicators of ineffective erythropoiesis. But unlike reactive processes, in which the pattern of ineffective erythropoiesis due to intramedullary hemolysis erythrokaryocytes, in this case it is a tumor lesion of red sprout and determined it a violation of his differentiation.
Diagnosing chronic erythremic myelosis
In normal morphology erythrokaryocytes evaluation process as a tumor difficult, which is characteristic for the diagnosis of many forms of chronic myeloid leukemia. Only unmotivated red sprout hyperplasia in the bone marrow, normocytosis blood, not due to any hemolysis, Our reparatsiey, indicate the nature of the tumor changes.
A supporting role in the diagnosis of chronic erythremic myelosis plays chromosome analysis, allowing to detect the presence of clone among myeloid cells, although specific to erythremic myelosis cytogenetic marker is not detected. Cytochemical erythrokaryocytes in this process can be characterized by a positive reaction to α-naftilesterazu, not completely suppress sodium fluoride, PAS-positive reaction, diffuse reaction to acid phosphatase. All these non-specific signs, erythrokaryocytes typical hyperplasia of red sprout as the tumor, and reactive nature.
Examination of the patient with chronic erythromyelosis reveals an enlarged spleen, which may be considerable. The spleen is detected punctate myeloid metaplasia with plenty erythrokaryocytes.
In the terminal stage of the process is characterized blastosis in the bone marrow and blood, sarcomatous increase in the lymph nodes, spleen or other organs.
