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Myelophthisic anemia |
Classification and external resources |
ICD-10 |
D61.9 |
ICD-9 |
284.2 |
eMedicine |
med/1562 |
MeSH |
D000750 |
Myelophthisic anemia (or myelophthisis) is a severe kind of anemia found in some people with diseases that affect the bone marrow. Myelophthisis refers to the displacement of hemopoietic bone-marrow tissue into the peripheral blood,[1] either by fibrosis, tumors or granulomas.
Contents
- 1 Causes
- 2 Diagnosis
- 3 Treatment
- 4 Presentation
- 5 Etiology
- 6 See also
- 7 References
Causes[edit]
Myelophthisis can occur in the setting of chronic myeloproliferative disease (e.g. myelofibrosis), 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[edit]
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 (dacryocytes), 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[edit]
Treatment of this disorder involves treatment of the underlying cancer.[2][3]
Presentation[edit]
Historically, the most common displacement of the healthy bone marrow was from tuberculosis.[citation needed]
There may be evidence of extramedullary hematopoiesis[4] (marrow elements can be found in the spleen, liver).
Etiology[edit]
Myelophthisis is thought to be related to the release of cytokines that simulate fibroblastic proliferation and fibrosis in the marrow.[2]
See also[edit]
- List of circulatory system conditions
- List of hematologic conditions
References[edit]
- ^ "Hematopathology".
- ^ a b c American Society of hematology self-assessment program, second edition, 2005, page 82.
- ^ "Myelophthisic Anemia: Anemias Caused by Deficient Erythropoiesis: Merck Manual Professional". Retrieved 2008-03-08.
- ^ Makoni SN, Laber DA (May 2004). "Clinical spectrum of myelophthisis in cancer patients". Am. J. Hematol. 76 (1): 92–3. doi:10.1002/ajh.20046. PMID 15114608.
Pathology: hematology, hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287)
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Red
blood cells |
↑ |
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↓ |
Anemia |
Nutritional |
- Micro-: Iron deficiency anemia
- Macro-: Megaloblastic anemia
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Hemolytic
(mostly Normo-) |
Hereditary |
- enzymopathy: G6PD
- glycolysis
- hemoglobinopathy: Thalassemia
- Sickle-cell disease/trait
- HPFH
- membrane: Hereditary spherocytosis
- Minkowski-Chauffard syndrome
- Hereditary elliptocytosis
- Southeast Asian ovalocytosis
- Hereditary stomatocytosis
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Acquired |
- Drug-induced autoimmune
- Drug-induced nonautoimmune
- Hemolytic disease of the newborn
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Aplastic
(mostly Normo-) |
- Hereditary: Fanconi anemia
- Diamond–Blackfan anemia
- Acquired: PRCA
- Sideroblastic anemia
- Myelophthisic
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Blood tests |
- MCV
- Normocytic
- Microcytic
- Macrocytic
- MCHC
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Other |
- Methemoglobinemia
- Sulfhemoglobinemia
- Reticulocytopenia
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Coagulation/
coagulopathy |
↑ |
Hyper-
coagulability |
- primary: Antithrombin III deficiency
- Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden
- Prothrombin G20210A
- Sticky platelet syndrome
- acquired:Thrombocytosis
- DIC
- Congenital afibrinogenemia
- Purpura fulminans
- autoimmune
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|
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↓ |
Hypo-
coagulability |
Thrombocytopenia |
- Thrombocytopenic purpura: ITP
- TM
- Heparin-induced thrombocytopenia
- May-Hegglin anomaly
|
|
Platelet function |
- adhesion
- aggregation
- Glanzmann's thrombasthenia
- platelet storage pool deficiency
- Hermansky–Pudlak syndrome
- Gray platelet syndrome
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Clotting factor |
- Hemophilia
- von Willebrand disease
- Hypoprothrombinemia/II
- XIII
- Dysfibrinogenemia
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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UpToDate Contents
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English Journal
- Bone Marrow Metastases from a 1p/19q Co-deleted Oligodendroglioma - A Case Report.
- Demeulenaere M1, Duerinck J2, DU Four S2, Fostier K3, Michotte A4, Neyns B5.
- Anticancer research.Anticancer Res.2016 Aug;36(8):4145-9.
- BACKGROUND: Metastasis of oligodendroglioma outside of the central nervous system (CNS) is a very rare event.CASE: We describe in detail the clinical story of a 50-year-old woman diagnosed with profound pancytopenia and signs of extramedullary hematopoiesis caused by diffuse bone marrow replacement
- PMID 27466523
- A Patient With Pancytopenia, Intractable Epistaxis, and Metastatic Prostate Cancer: How Correct Diagnosis of Primary Hyperfibrinolysis Helps to Stop the Bleeding.
- Jafri MA1, Cohen JV2, Much MA3, Petrylak DP4, Podoltsev NA5.
- Clinical genitourinary cancer.Clin Genitourin Cancer.2016 May 27. pii: S1558-7673(16)30128-8. doi: 10.1016/j.clgc.2016.05.002. [Epub ahead of print]
- PMID 27320762
- Metastatic Breast Cancer with Extensive Osseous Metastasis Presenting with Symptomatic Immune Thrombocytopenic Purpura and Anemia: A Case Report and Review of the Literature.
- Niu J1, Goldin T2, Markman M3, Kundranda MN1.
- Case reports in oncology.Case Rep Oncol.2015 Jun 5;8(2):256-63. doi: 10.1159/000431213.
- BACKGROUND: Immune thrombocytopenic purpura (ITP) is a rare acquired bleeding disorder with an estimated incidence of 1 in 10,000 people in the general population. The association of ITP with breast cancer is an even rarer entity with very limited reports in the English literature.CASE PRESENTATION:
- PMID 26120311
Japanese Journal
- Successful chemo-endocrine therapy for multiple bone metastases and myelophthisis caused by occult breast carcinoma
- International journal of clinical oncology : official journal of the Japan Society of Clinical Oncology 5(6), 399-404, 2000-12-01
- NAID 10009600137
- 白赤芽球症について 定義の確認と臨床的重要性:―定義の確認と臨床的重要性―
Related Links
- myelophthisis /my·e·loph·thi·sis/ (mi″ĕ-lo-thi?sis) 1. wasting of the spinal cord. 2. bone marrow suppression secondary to marrow infiltration by tumor with local production of myelosuppressive cytokines. my·e·loph·thi·sis (mī′ə-lŏf′thĭ ...
- myelophthisis my·e·loph·thi·sis (mī'ə-lŏf'thĭ-sĭs, -lō-tī'-,) n. Wasting or atrophy of the spinal cord. Replacement of hemopoietic tissue in the bone marrow by abnormal tissue, usually fibrous tissue or malignant tumors. Also called panmyelophthisis
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