leucopenia and familial mediterranean fever
TRANSCRIPT
Pediatric Hematology and Oncology, 31:129–130, 2014Copyright C© Informa Healthcare USA, Inc.ISSN: 0888-0018 print / 1521-0669 onlineDOI: 10.3109/08880018.2013.800928
LETTER TO THE EDITORNeutropenia and Bone Marrow Failure
Leucopenia and Familial Mediterranean Fever
Kostas Konstantopoulos, MD
Medicine I, Athens University, Athens, Greece
Keywords genetics, leukocytes, neutropenia
I would like to comment on the paper by Aslan on leukopenia on FMF (FamilialMediterranean Fever) by Aslan [1]. According to this paper, FMF causes leukopeniamainly due to a decrease of neutrophils which is a consequence of autophagy andapoptosis.
Aslan’s study is based on evaluation of leukopenia in 15 Turkish cases that eventu-ally were also diagnosed as FMF. It is rather clear that these patients did not alreadycarry an FMF diagnosis established and that further molecular FMF investigation wasconducted on the occasion of hematologic consultation. Thus, all his leukopenia caseswere newly diagnosed. In this case, it would be important to know as to how manycases were investigated for leukopenia during the same period from the same areaand the underlying reason(s) found. This is an important issue as Turkey is a rather“hot” FMF area. If a lot FMF cases exist in a certain area, the possibility for coexis-tence of another disease or condition should always be considered [2]. One should alsotake in mind that the role of MEFV mutation genes in “third” conditions like colitis,Behcet’s disease is a controversial matter and that all putative “pathophysiologicalrole” of pyrin in all other conditions, must be interpreted with caution. To this effect,data from countries less affected by MEFV gene mutations may be more informative.
Furthermore, in order to evaluate any “primary” effect of pyrin mutations on whitecell counts and kinetics, one should extend the investigation to the so-called “pheno-type III” Mediterranean Fever, i.e., asymptomatic homozygotes not expressing a clin-ical phenotype [3, 4].
Fluctuations of white cell numbers in “recurrent polyserositis” are long known. Ofinterest, clinicians think of a notable association between cyclic neutropenia and pa-tients suffering of what today is known as FMF [5, 6]; however, some others note leuko-cytosis [7]. Of interest, most recent papers on inflammation in FMF cases and FMFcarriers, refer to markers like CRP, ESR, and SAA but not white cell counts [8].
In our department, we have typed a total of 87 cases for an established diagnosis ofFMF. We noted no case of leukopenia before commencement of colchicine therapy;
Received 14 April 2013; accepted 26 April 2013; published online 2014.The author thanks Alexandra Kanta for her helpful contribution.The recent paper by Aslan in Pediatric Hematology and Oncology is commented.Address correspondence to Professor Kostas Konstantopoulos, MD, Athens University, Medicine I,75, MIkras Asias Str, Athens, 11527 Greece. E-mail: [email protected]
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following colchicine, a degree of lower white cell counts were encountered, but nocase approaching levels of concern was noted. Notably, our cases are mainly adults ofGreek origin.
In our experience, it is difficult to claim any effect of pyrin and pyrin mutations onwhite cell number /leukopenia. A nice idea may be a search for MEFV mutations oncyclic neutropenia cases, in a population with a known (low) frequency of MEFV.
Declaration of Interest
The author reports no conflict of interest. The author alone is responsible for the con-tent and writing of the paper.
This work is mainly supported by Athens University Grands Fund.
REFERENCES
[1] Aslan D. Leukopenia in familial Mediterranean fever: case series and literature review with spe-cial emphasis on pathogenesis. Pediatr Hematol Oncol. 2013;[Epub ahead of print] PubMed PMID:23560861.
[2] Ben-Chetrit E, Yazici H. Thoughts on the proposed links between Behcet’s disease and familialMediterranean fever. Clin Exp Rheumatol. 2002;20(4 Suppl 26):S1–S2. PubMed PMID: 12371627.
[3] Kogan A, Shinar Y, Lidar M, Revivo A, et al. Common MEFV mutations among Jewish ethnic groupsin Israel: high frequency of carrier and phenotype III states and absence of a perceptible biologicaladvantage for the carrier state. Am J Med Genet. 2001;102(3):272–6. PubMed PMID: 11484206.
[4] Konstantopoulos K. Endocrine function and dysfunction in familial Mediterranean fever. ClinRheumatol. 2006;25(6):885. Epub 2006 Jul 7. PubMed PMID: 16496077.
[5] Siguier F, Sebaoun J. Periodic disease with attacks of sialorrhea and cyclic neutropenia. Bull Mem SocMed Hop Paris. 1954;70(1–2):27–31. PubMed PMID: 13150179.
[6] Reimann HA. Periodic disease; periodic fever, periodic abdominalgia, cyclic neutropenia, intermit-tent arthralgia, angioneurotic edema, anaphylactoid purpura and periodic paralysis. J Am Med Assoc.1949;141(3):175–83. PubMed PMID:18139542.
[7] Priest RJ, Nixon RK. Familial recurring polyserositis: a disease entity. Ann Intern Med.1959;51:1253–1274. PubMed PMID: 14434876.
[8] Lachmann HJ, Sengul B, Yavuzsen TU, et al. Clinical and subclinical inflammation in patients with fa-milial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford).2006;45(6):746–50. PubMed PMID: 16403826.
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