Myelophthisis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Soumya Sachdeva
Synonyms and keywords: Myelophthisic anemia
Overview
Myelophthisis refers to the displacement of hemopoietic bone-marrow tissue into the peripheral blood, either by fibrosis (myelofibrosis), tumors, or granulomas. A myelophthisic blood smear is one which contains nucleated red blood cells, granulocyte precursors, and teardrop-shaped erythrocytes. This condition is also known as Myelophthisic anemia. Myelophthisis can be observed in cancers that that involve the bone marrow such as leukemia, lymphoma, and myeloma or metastatic carcinoma. The commonest cause is metastatic cancer, most often carcinomas arising in breast, lung and prostate.However any infiltrative process (e.g.,granulomatous disease) involving the marrow can produce identical findings.It should be remembered that it is also a feature of spent phase of myeloproliferative disorders.They cause marrow distortion and fibrosis which act to displace normal marrow elements and disturb mechanisms that regulate the egress of red cells and granulocytes from the marrow.
Historical Perspective
Classification
Pathophysiology
Myelophthisis is thought to be caused by damage to the normal hematopoietic stem cells, which leads to the release of cytokines, growth factors and other substance. Immature cells, stem cells and progenitor cells are thus released and relocate in the spleen and the liver .
Causes
Differentiating Myelophthisis from Other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications, and Prognosis
Natural History
Complications
[1] The complications are related to pancytopenia including anemia and its complications , infections and the bleeding tendency in addition to the hypersplenism which leads to refractory thrombocytopenia and portal hypertension.
Prognosis
The prognosis of Myelophthisis depends on treating the etiology
Diagnosis
Diagnostic Criteria
History and Symptoms
patients usually complain of symptoms of palpitations , fatigue , dyspnea and weakness .
Physical Examination
[1] patients will show signs of anemia such as pale conjunctiva , enlarged spleen and tachycardia. In addition they will likely have increased tendency to bleed and susceptibility to infections.
Laboratory Findings
Peripheral blood picture will show :
- normocytic normochromic anemia .
- Increased RBCs count.
- leukoerthroblasts
- Abnormally shaped RBCs.
- Bone marrow is often confirmatory ; will likely show dry tap due to the replacement of normal cells by fibrosis and abnormal cells which will aid in the diagnosis of the underlying etiology .
Imaging Findings
Other Diagnostic Studies
Treatment
Treatment of Myelophthisis depends on treating the underlying cause.