Myelophthisic Anemia

Myelophthisic Anemia

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Myelophthisis is a form of bone marrow failure that results from the destruction of bone marrow precursor cells and their stroma, which nurture these cells to maturation and differentiation. (See Etiology.)

Infiltrating lesions caused by nonhematopoietic cells invading bone marrow can result in varying degrees of cytopenia, including anemia, thrombocytopenia, neutropenia, and pancytopenia. [1]  Bone marrow failure resulting from secondary infiltration is a possible cause of lack of blood cell production (as differentiated from a primary cause of failure). Manifestations range from a leukoerythroblastic picture [2]  to the presence of a few teardrop-shaped red blood cells and early myeloid precursor cells in the peripheral blood smear. [3] (See the image below.) (See Etiology, Differentials, and Workup.)

The most common causes of infiltrative myelopathy are metastatic carcinomas (eg, lung, breast, and prostate cancer), [4, 5]  lymphoproliferative malignancies (eg, lymphomas), disseminated granulomatous diseases (eg, miliary tuberculosis), [6] and rare diseases (eg, Gaucher disease). (See the image below.) (See Etiology.)

Go to AnemiaChronic AnemiaMegaloblastic AnemiaHemolytic Anemia, and Sideroblastic Anemias for complete information on these topics.

Generally, in myelophthisic anemia, a form of fibrosis, occurs secondary to injury by nonhematopoietic cells or pathogens. This fibrosis destroys the normal hematopoietic cells and their supportive stromal cells. The bone marrow becomes infiltrated by collagen, reticulin, and other forms of fibrosis, which replace the normal, hematopoietic cells. The most common causes of extensive bone marrow infiltrative damage or invasion without much structural damage are discussed below. The expanding number and volume of pathologic cells and the release of suppressive cytokines can eventually lead to bone marrow failure without the characteristic morphologic features of myelophthisis.

Leukemic cells, such as those occurring in chronic leukemias in which the expanding cells are mature and coexist peacefully with the normal bone marrow cells, show no evidence of myelophthisis, and marrow damage does not occur.

In agnogenic and secondary myelofibrotic disorders, megakaryocytes release platelet-derived growth factors, which are fibroblastic stimulants for growth and proliferation. This leads to the consequences of bone marrow space reduction and to disruption of normal bone marrow architecture.

Agnogenic myeloid metaplasia is a stem cell abnormality associated with myeloproliferative diseases. It is related to an abnormal stem cell clone that stimulates increased myelofibrosis and damage. It progresses to acute leukemia and is associated with extramedullary hematopoiesis in the liver and spleen, causing hypertrophy of these organs.

Secondary myelofibrosis is due to implantation or invasion by malignant cancer cells that have metastasized because of implantation of blood-borne tumor cells from a distant cancer. The most common sources are cancers of the lung, breast, and prostate [7] and sarcomas.

Nonmalignant causes of myelophthisis include the following:

Inflammatory cells, miliary tuberculosis, and fungal infections

Sarcoidosis

Macrophage proliferation in storage diseases, such as Gaucher disease

Necrosis in sickle cell disease and septicemia

Bone disease in congenital osteopetrosis

With regard to Gaucher disease, infiltration by cells with “onion-peel” cytoplasm, called Gaucher cells, is caused by a lipid storage disorder (ie, glucosylceramide lipidosis). Gaucher cells clog or infiltrate the bone marrow, spleen, and liver. This disorder is inherited.

Myelophthisis is observed more frequently in countries where access to medical care is difficult and diseases are allowed to progress to advanced stages. In the United States, infiltrative myelopathy occurs in less than 10% of cancer patients with metastatic disease.

Mortality is dependent on the underlying condition. [5] The leukoerythroblastic blood picture is often associated with imminent death in some extreme cases. Patients with varying degrees of cytopenia are at risk for infection or bleeding.

Pham CM, Syed AA, Siddiqui HA, Keller RA, Kowalewski C. Case of metastatic basal cell carcinoma to bone marrow, resulting in myelophthisic anemia. Am J Dermatopathol. 2013 Apr. 35 (2):e34-6. [Medline].

Delsol G, Guiu-Godfrin B, Guiu M, Pris J, Corberand J, Fabre J. Leukoerythroblastosis and cancer frequency, prognosis, and physiopathologic significance. Cancer. 1979 Sep. 44(3):1009-13. [Medline].

Mahdi EJ, Mahdi AJ. Leucoerythroblastosis and thrombocytopenia as clues to metastatic malignancy. BMJ Case Rep. 2014 Jan 31. 2014:[Medline]. [Full Text].

Brochamer WL Jr, Keeling MM. The bone marrow biopsy, osteoscan, and peripheral blood in non-hematopoietic cancer. Cancer. 1977 Aug. 40(2):836-40. [Medline].

Makoni SN, Laber DA. Clinical spectrum of myelophthisis in cancer patients. Am J Hematol. 2004 May. 76(1):92-3. [Medline].

Bodem CR, Hamory BH, Taylor HM, Kleopfer L. Granulomatous bone marrow disease. A review of the literature and clinicopathologic analysis of 58 cases. Medicine (Baltimore). 1983 Nov. 62(6):372-83. [Medline].

Shamdas GJ, Ahmann FR, Matzner MB, Ritchie JM. Leukoerythroblastic anemia in metastatic prostate cancer. Clinical and prognostic significance in patients with hormone-refractory disease. Cancer. 1993 Jun 1. 71(11):3594-600. [Medline].

Sharma P, Pati HP, Mishra PC, et al. Inability of immunomorphometric assessment of angiogenesis to distinguish primary versus secondary myelofibrosis. Anal Quant Cytol Histol. 2011 Aug. 33(4):236-44. [Medline].

Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19. 127 (20):2391-405. [Medline]. [Full Text].

Rosner S, Sen F, Postow M. Response after treatment with pembrolizumab in a patient with myelophthisis due to melanoma: the role of checkpoint inhibition in the bone. J Immunother Cancer. 2017. 5:34. [Medline]. [Full Text].

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Koyamangalath Krishnan, MD, FRCP, FACP Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, Royal College of Physicians

Disclosure: Nothing to disclose.

Myelophthisic Anemia

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