Polycythemia historical perspective: Difference between revisions
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===Discovery=== | ===Discovery=== | ||
*Polycythemia vera was first mentioned in 1892 in the medical literature by a French physician Louis Henri Vaquez. | *Polycythemia vera was first mentioned in 1892 in the medical literature by a French physician Louis Henri Vaquez. <ref name="urlThe history of myeloproliferative disorders: before and after Dameshek | Leukemia">{{cite web |url=https://www.nature.com/articles/2404946#Sec6 |title=The history of myeloproliferative disorders: before and after Dameshek | Leukemia |format= |work= |accessdate=}}</ref> | ||
*In 1899 and 1900 two additional cases of polycythemia vera were described by Richard Clark Cabot both with increased erythrocytes and leukocytes and one with marked splenomegaly. | *In 1899 and 1900 two additional cases of polycythemia vera were described by Richard Clark Cabot both with increased erythrocytes and leukocytes and one with marked splenomegaly. | ||
Revision as of 23:55, 3 December 2020
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Polycythemia Microchapters |
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Treatment |
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Polycythemia historical perspective On the Web |
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Risk calculators and risk factors for Polycythemia historical perspective |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Debduti Mukhopadhyay, M.B.B.S[2]
Overview
Historical Perspective
Discovery
- Polycythemia vera was first mentioned in 1892 in the medical literature by a French physician Louis Henri Vaquez. [1]
- In 1899 and 1900 two additional cases of polycythemia vera were described by Richard Clark Cabot both with increased erythrocytes and leukocytes and one with marked splenomegaly.
- In 1951, William Dameshek grouped together chronic myelogenous leukemia (CML), polycythemia vera, essential thrombocythemia, primary myelofibrosis, and erythroleukemia and coined the term "myeloproliferative disorders". [2]
- In 2005, a gain of function mutation in JAK2 (JAK2V617F) was first implicated in the pathogenesis of BCR-ABL negative myeloproliferative disorders. A liquid culture system was used to culture PV erythroid cells without exogenous cytokines. it was noted that PV erythroid proliferation was inhibited with inhibition of JAK2.
- Subsequently siRNA was used to prove that JAK2 expression was necessary for the expansion of erythrocytes in the absence of cytokines, which led to the sequencing of JAK2 in PV patients.
- The JAK2V617F mutation is responsible for creating instability in the chromatin structure of DNA, thus reducing the response to apoptosis.
Landmark Events in the Development of Treatment Strategies
- In 1960, Peter Nowell and David Hungerford published data on an abnormally small chromosome which looked like a Y chromosome, the data came from two male patients with CML. Eventually seven more cases were discovered, with the presence of the specific chromosomal abnormality.
- Nowell and Hungerford further noticed that these abnormal cells coexisted with normal karyotype, and, thus concluded that the abnormally small chromosome might be a cause of CML rather than coincidental.
- This abnormally small chromosome was named as the Philadelphia chromosome, after the city it was discovered in.
- Polycythemia Vera Study Group : Louis Wasserman in 1967 created a group with clinicians from all over the country to study PV in detail. Major significance of this was to study the leukemogenicity of radioactive phosphorus which was one of the major agents used at that time for the treatment of PV.
- Prior to this, the mainstay of treatment were phlebotomy and IV P-32. Other modalities included the following : skeletal radiation therapy, acetylphenylhydrazine, potassium arsenite, lead acetate, nitrogen mustard, hydroxyurea, melphalan, etc.
Famous Cases
The following are a few famous cases of [disease name]:
- Phyllis George - A former Miss America and sportscaster, passed away at age 70, due to complications from Polycythemia vera on May, 14, 2020.
References
- ↑ "The history of myeloproliferative disorders: before and after Dameshek | Leukemia".
- ↑ Tefferi A (January 2008). "The history of myeloproliferative disorders: before and after Dameshek". Leukemia. 22 (1): 3–13. doi:10.1038/sj.leu.2404946. PMID 17882283.