Prefibrotic primary myelofibrosis

Ntụnyere na myleofibrosis mbụ dezie

Reticulin ma ọ bụ colajin Fibrosis ogo abụọ ma ọ bụ atọ bụ usoro nchọpụta maka myelofibrosis bụ isi.

Ntụnyere na Thrombocythemia dị mkpa dezie

Ma pre-PMF na uru thrombocythemia nwere ike ịkekọrịta ihe nchọpụta, dị ka mmụba nke megakaryocytes na ọnụnọ nke mutation. Ọnụnọ nke Reticulin Fibrosis na pre-PMF na-enye ọdịiche doro anya n'etiti abụọ ahụ.

Ndị ọrịa a na-ewere na ha nwere obere ihe ize ndụ maka thrombosis ma ọ bụ nnukwu ọbara ọgbụgba kwesịrị ịhụ naanị. A na-atụ aro aspirin dị ala maka ndị ọrịa na-enweghị akụkọ ihe mere eme nke thrombosis. Maka ndị ọrịa nọ n'ihe ize ndụ dị gn'etiti, ọgwụgwọ na-akpali mgbaàmà maka anaemia ma ọ bụ mgbaàmà nke usoro.   [citation needed]

Maka ndị ọrịa nwere nnukwu ihe ize ndụ nwere akụkọ ihe mere eme nke trombosis, a na-atụ aro ọgwụ mgbochi ọnụ na ọgwụ cytoreductive dị ka hydroxycarbamide, a ga-emekwa onye ọrịa ahụ dịka ọ dị na myelofibrosis mbụ.[1][2]

Nkọwapụta nke pre-PMF ugbu a na-ata ahụhụ site na _enweghị ozi ọtụtụ etiti, yana ọtụtụ ajọ mbunobi sitere na ọrịa ahụ dị iche na reclassification nke WHO n'afọ 2016. Afọ iri nke mmụba na PMF doro anya dị n'etiti 9.7 na pasenti iri atọ n'ime ise. Afọ iri nke mgbanwe n'ime ọrịa leukemia siri ike sitere na 5.8% ruo pesenti iri na abụọ [3]

Ihe nlereanya nke akara ngosi nke e mere kpọmkwem maka pre-PMF adịbeghị, mana e nwere ike iji International Prognostic Scoring System mee ihe iji buo amụma na ndị ọrịa pre-PMf ga-adị ndụ.[4] Nnyocha ọtụtụ ụlọ ọrụ na ndị ọrịa PMF a gbanwere aha ha gosipụtara ọkara ndụ nke ndị ọrịa pre-PMF na afọ 17.6 ma e jiri ya tụnyere afọ 7.2 maka ndị ọrịa PMP doro anya.[1] Otú ọ dị, nnyocha ọzọ gosiri na 98% nke ndị ọrịa pre-PMF dị ndụ mgbe afọ 10 gasịrị site na nchọpụta na mgbe ndị ọrịa PMF doro anya gosipụtara ọkara ndụ nke afọ 16.6, ọ dịghị ike ịgbakọ ọkara ndụ pre-PMf dịka ihe karịrị pasenti iri ise nke ndị ọrịa ka dị ndụ n'oge mbipụta.[5]

Akụkọ ihe mere eme dezie

Nke mbụ akọwapụtara na 1976, e webatara pre-PMF n'ime nhazi nke WHO nke akpụ ruo n'afọ 2001, ọ bụghịkwa nke a na-ekewa dị ka ihe dị iche ruo mgbe e dezigharịrị ya na 2016.[1]

  1. 1.0 1.1 1.2 Finazzi (2018-11-07). "Prefibrotic myelofibrosis: treatment algorithm 2018" (in en). Blood Cancer Journal 8 (11). DOI:10.1038/s41408-018-0142-z. ISSN 2044-5385. PMID 30405096. Finazzi, Guido; Vannucchi, Alessandro M.; Barbui, Tiziano (2018-11-07). "Prefibrotic myelofibrosis: treatment algorithm 2018". Blood Cancer Journal. 8 (11): 104. doi:10.1038/s41408-018-0142-z. ISSN 2044-5385. PMC 6221891. PMID 30405096. Kpọpụta njehie: Invalid <ref> tag; name "treatmentalgo" defined multiple times with different content
  2. Barbui (2019). "Comments on pre-fibrotic myelofibrosis and how should it be managed" (in en). British Journal of Haematology 186 (2): 358–360. DOI:10.1111/bjh.15840. ISSN 1365-2141. PMID 30847907. 
  3. Barbui (2019). "Comments on pre-fibrotic myelofibrosis and how should it be managed" (in en). British Journal of Haematology 186 (2): 358–360. DOI:10.1111/bjh.15840. ISSN 1365-2141. PMID 30847907. 
  4. Rumi (2018-07-03). "Diagnosis and management of prefibrotic myelofibrosis". Expert Review of Hematology 11 (7): 537–545. DOI:10.1080/17474086.2018.1484280. ISSN 1747-4086. PMID 29862872. 
  5. Barosi (2012-04-20). "Evidence that Prefibrotic Myelofibrosis Is Aligned along a Clinical and Biological Continuum Featuring Primary Myelofibrosis" (in en). PLOS ONE 7 (4): e35631. DOI:10.1371/journal.pone.0035631. ISSN 1932-6203. PMID 22536419.