Osteomalaciei

Osteomalaciei (osteomyelodysplasia; from the Greek. osteon - bone, myelos - brain + dysplasia, synonym: osteosclerotic leukemia, anemia, myelofibrosis) -a disease characterized by the combination sclerosis bones with changes in the bone marrow and blood.
In relation to the pathogenesis of the disease there is no consensus. Allow belonging to the group of leukemia. However Century A. Rusakov believes that the basis of osteomalaciei is a violation correlation between bone and blood-forming tissues.
Osteomalaciei described under various (more than 30) names. The disease is flowing slowly (10-20 years or more)is observed more often in old age; one of the first signs is the enlargement of the spleen. Acting almost always accompanied by a progressive anemia (see), often a symptom of erythremia. In cases proceeding with the picture mieloza, leukocytosis rarely exceeds 20 000-50 000, and in the blood are promyelocyte, immature cells of erythropoiesis, a lot of plasmic order has been revealed.
The constant companion of osteomalaciei is extramedullary hematopoiesis in the spleen, liver, lymph nodes, sometimes also in other organs (Fig. 1). In sometimes increased to 4-6 kg spleen note stromal fibrosis and pockets of blood often with a large number of megakaryocytes.
When bone marrow bone pierced with difficulty. In the received punctate bone marrow find Mature and immature cells of all three germ of hemopoiesis.
Radiographically in the bones revealed sclerotic changes, covering, in varying degrees in almost all the bones of the skeleton. The external shape of the bone remains, only occasionally observed periosteal layering on the tubular bones. On the cuts - spongy bone with or diffuse alopecia sealed beam structures (Fig. 2).
The osteosclerosis caused by the growth of new bone beams, forming a dense network in the bone marrow spaces (Fig. 3), and a thickening of old beams due to the layering of them new bone tissue. Medullar canal are often filled with spongy bone structures. There is a continuous restructuring of bones with the prevalence of bone formation and less prominent resorption process beams and formation of cavities, which sometimes leads to spongicola compact bone. The newly formed bone has an atypical structure. Preserved bone marrow space at a significant during filled with fibrous tissue angosta. Among this fibrous tissue scattered pockets of blood cells eritro-, mielo - and trombopenia in different degree of their maturity.
Radical treatment of osteomalaciei not. Splenectomy is allowable only in exceptional cases (excessive enlargement of the spleen). Cm. Leukemia, Osteodysplastic.

Fig. 1. Pockets of extramedullary hematopoiesis with numerous megakaryocytes in the spleen.
Fig. 2. Seal, thickening of bone structures of spongiosa vertebral body (above). Pronounced osteosclerosis ribs (bottom).
Fig. 3. A dense network of newly formed bone beams, layering on old structures. Notes uneven their crashevenmore. Pockets of blood (1) on the background of fibrous tissue.