Osteomyelosclerosis

When anemia accompanied by moderately elevated (although sometimes reduced) leukocyte counts, thrombocytopenia or thrombocytosis, clinically evident splenic tumor, left shift up to and including sporadic myeloblasts, and eosinophilia, the presence of a large proportion of red cellpre-cursors (normoblasts) in the differential blood analysis, osteomyelosclero-sis should be suspected. BCR-ABL gene analysis is negative.

Pathologically, osteomyelosclerosis usually originates from megakaryocyte neoplasia in the bone marrow and the embryonic hematopoietic organs, particularly spleen and liver, accompanied by fibrosis (= sclerosis) that will eventually predominate in the surrounding tissue. The central role of cells of the megakaryocyte series is seen in the giant thrombocytes, or even small coarsely structured megakaryocyte nuclei without cytoplasm, that migrate into the blood stream and appear in the CBC. OMS can be a primary or secondary disease. It may arise during the course of other myeloproliferative diseases (often polycythemia vera or idiopathic thrombocythemia).

Tough, fibrous material hampers the sampling of bone marrow material, which rarely yields individual cells. This in itself contributes to the bone marrow analysis, allowing differential diagnosis versus reactive fibroses (parainfectious, paraneoplastic).

Characteristics of OMS

Age of onset: Usually older than 50 years.

Clinical findings: Signs of anemia, sometimes skin irritation, drastically enlarged spleen.

CBC: Usually tricytopenia, normoblasts, and left shift. Further diagnostic procedures: Fibrous bone marrow (bone marrow histology), when appropriate and BCR-ABL (always negative). Differential diagnosis: Splenomegaly in cases of lymphadenoma or other myeloproliferative diseases: bone marrow analysis. Myelofibrosis in patients with metastatic tumors or inflammation: absence of splenomegaly.

Course, therapy: Chronic disease progression; transformation is rare. If there is splenic pressure: possibly chemotherapy, substitution therapy.

Further myeloproliferative diseases are described together with the relevant cell systems: polycythemia vera (see p. 162) and essential throm-bocythemia (see p. 170).

Enlarged spleen and presence of immature white cell precursors in peripheral blood suggest osteomyelosclerosis a c

Megakaryocytes Nucleus

Fig. 42 Osteomyelosclerosis (OMS). a and b Screening of blood cells in OMS: red cell precursors (orthochromatic erythroblast = 1 and basophilic erythroblast = 2), basophilic granulocyte (3), and teardrop cells (4). c Sometimes small, dense megakaryocyte nuclei are also found in the blood stream in myeloproliferative diseases. d Blast crisis in OMS: myeloblasts and segmented basophilic granulo-cytes (1).

Fig. 42 Osteomyelosclerosis (OMS). a and b Screening of blood cells in OMS: red cell precursors (orthochromatic erythroblast = 1 and basophilic erythroblast = 2), basophilic granulocyte (3), and teardrop cells (4). c Sometimes small, dense megakaryocyte nuclei are also found in the blood stream in myeloproliferative diseases. d Blast crisis in OMS: myeloblasts and segmented basophilic granulo-cytes (1).

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