Myelophthisic anemia

Myelophthisic anemia (or myelophthisis) is a severe kind of anemia found in some people with diseases that affect the bone marrow. Myelophythisis refers to the displacement of hemopoietic bone-marrow tissue into the peripheral blood,^[1] either by fibrosis, tumors or granulomas.

Causes

Myelophythisis can occur in the setting of chronic myeloproliferative disoders, leukemia, lymphoma, and metastatic carcinoma or myeloma. It is common in people who have chronic idiopathic myelofibrosis. It has been linked to small-cell lung cancer, breast cancer or prostate cancer that metastasizes to the bone marrow.^[2]

Diagnosis

The first test for diagnosis myelophthisis involves looking at a small sample of blood under a microscope. Myelophthisis is suggested by the presence of red blood cells that contain nuclei or are teardrop-shaped (poikilocytotic cells), or immature granulocyte precursor cells which indicates leukoerythroblastosis is occurring because the displaced hematopoietic cells begin to undergo extramedullary hematopoiesis. These immature granulocytes are seen in peripheral blood smears. Diagnosis is confirmed when a bone marrow biopsy demonstrates significant replacement of the normal bone marrow compartment by fibrosis, malignancy or other infiltrative process. The presence of immature blood cell precursors helps distinguish another cause of pancytopenia, aplastic anemia, from myelophthisic anemia because in aplastic anemia the hematopoietic cells are damaged and immature blood cells are not seen in the peripheral blood.

Treatment

Treatment of this disorder involves treatment of the underlying cancer.^[3][4]

Presentation

Historically, the most common displacement of the healthy bone marrow was from tuberculosis.^{[citation needed]}

There may be evidence of extramedullary hematopoiesis^[5] (marrow elements can be found in the spleen, liver).

Etiology

Myelophthisis is thought to be related to the release of cytokines that simulate fibroblastic proliferation and fibrosis in the marrow.^[6]

References

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