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==Background== | ==Background== | ||
*Chronic myeloproliferative disorder | *Chronic [[myeloproliferative disorders|myeloproliferative disorder]] | ||
*Abnormal proliferation is seen in all 3 cell lines | *Abnormal proliferation is seen in all 3 cell lines | ||
*Typically in elderly | *Typically in elderly | ||
*Can be asymptomatic or symptomatic | *Can be asymptomatic or symptomatic | ||
*Can be primary or develop secondary to [[polycythemia vera]] or [[essential thrombocytosis]] | |||
==Clinical Features== | ==Clinical Features== | ||
*Palpable splenomegaly | |||
*Bone pain | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Polycythemia vera]] (PV) | *[[Polycythemia vera]] (PV) | ||
*Essential thrombocytosis (ET) | *[[Essential thrombocytosis]] (ET) | ||
*[[Chronic myelogenous leukemia]] (CML) | *[[Chronic myelogenous leukemia]] (CML) | ||
*Leukemoid | *[[Leukemoid Reaction]] | ||
==Evaluation== | ==Evaluation== | ||
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*Bone marrow biopsy | *Bone marrow biopsy | ||
=== | ===Diagnosis<ref>Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,</ref>=== | ||
*Criteria: The diagnosis of | *[[Anemia]] | ||
*[[Pancytopenia]] (in late disease) | |||
*Increased serum LDH | |||
*Criteria: The diagnosis of primary myelofibrosis requires meeting all 3 major criteria and 2 minor criteria. | |||
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==Management== | ==Management== | ||
*Blood transfusion | *[[Blood transfusion]] | ||
*Ruxolitinib | *Ruxolitinib | ||
*Allogeneic stem cell transplantation | *Allogeneic stem cell transplantation | ||
Revisión actual - 04:56 1 oct 2019
Background
- Chronic myeloproliferative disorder
- Abnormal proliferation is seen in all 3 cell lines
- Typically in elderly
- Can be asymptomatic or symptomatic
- Can be primary or develop secondary to polycythemia vera or essential thrombocytosis
Clinical Features
- Palpable splenomegaly
- Bone pain
Differential Diagnosis
- Polycythemia vera (PV)
- Essential thrombocytosis (ET)
- Chronic myelogenous leukemia (CML)
- Leukemoid Reaction
Evaluation
Workup
- CBC
- Blood smear
- Chem 7
- Bone marrow biopsy
Diagnosis[1]
- Anemia
- Pancytopenia (in late disease)
- Increased serum LDH
- Criteria: The diagnosis of primary myelofibrosis requires meeting all 3 major criteria and 2 minor criteria.
| Major criteria |
|---|
| Megakaryocyte proliferation and atypia with reticulin and/or collagen fibrosis,
or with increased marrow cellularity, granulocytic proliferation, and decreased erythropoiesis |
| Not meeting WHO criteria for CML, PV, MDS, or other myeloid neoplasm |
| Demonstration of JAK2V617F or other clonal marker or no evidence of reactive bone marrow fibrosis |
| Minor criteria |
|---|
| Leukoerythroblastosis |
| Increased serum LDH |
| Anemia |
| Palpable splenomegaly |
Management
- Blood transfusion
- Ruxolitinib
- Allogeneic stem cell transplantation
Also See
References
- ↑ Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,