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Neutropénies Idiopathiques de l’Adulte
Dr Flore Sicre de Fontbrune - Nantes - 22 septembre 2017
Neutropénie Idiopathique Chronique de l’Adulte
• PNN < 1,5 G/L
• > 3 mois
• Exclusion des formes secondaires
• Exclusion des formes auto-immunes primitives ie anticorps anti-granuleux
2
Définition Théorique ou Pratique ?
Neutropénie Idiopathique Chronique de l’Adulte
• PNN < 1,5 G/L
• > 3 mois
• Exclusion des formes secondaires
• Exclusion des formes auto-immunes primitives ie anticorps anti-granuleux
3
Définition Théorique ou Pratique ?
En pratique : • Rarement explorés si > 1 G/L • Délai de 3 mois uniquement dans les formes modérées ou sévères
asymptomatiques • Pas de test diagnostique pour les formes ethniques • Différences entre auto-immunes primitives et idiopathiques ?
Neutropénie Auto-Immune Primitive de l’Adulte
4
GIFT GAT
MAIGA
Neutropénie Auto-Immune Primitive de l’Adulte
5
Difficultés techniques
GIFT : PNN pts GAT : PNN donneur phénotypés
Faux positifs
Anti HLA I Complexes immuns
GCSF Grossesse
Faux négatifs
Faible sensibilité (titre/affinité)
MAIGA
Test + mais Ag non identifié
Neutropénie Auto-Immune Primitive de l’Adulte
6
Difficultés techniques
GIFT : PNN pts GAT : PNN donneur phénotypés
Faux positifs
Anti HLA I Complexes immuns
GCSF Grossesse
Faux négatifs
Faible sensibilité (titre/affinité)
MAIGA
Test + mais Ag non identifié
NEUTROPÉNIE AUTO-IMMUNES PRIMITIVES
& NEUTROPÉNIES IDIOPATHIQUES ADULTES
Epidémiologie similaire (adulte) Démarche diagnostique similaire
Prise en charge thérapeutique similaire Pas de données objectives pour les différencier
Neutropénie Idiopathique Chronique de l’Adulte
Epidémiologie = maladie rare
Dale D. – The severe Chronic Regsitry International Registry, 10 years FU - Supportive Cancer Therapy 2005
PNN < 0,5G/L, > 3 mois, non exhaustif - 1163 patients sur 35 pays
Nette prépondérance féminine
Neutropénie Idiopathique Chronique de l’Adulte
Epidémiologie
Dale D. – un update on the diagnosis and treatment avec CIN – Current opinion in Hematology 2017
CIN + AIN
Neutropénie Idiopathique Chronique de l’Adulte
Epidémiologie
Registre Français
1993-2014
2134 patients (tout patient signalé)
Sévère Modérée sympto
> 3 mois FU > 1 an
178 pts
Primitive
108
797 constitutionnelles Age médian 28 21;40
Femme 79%
NCSIA DÉMARCHE DIAGNOSTIQUE PNN < 1.5 G/L isolated
• Familial history
• Clinical evaluation
Clinical & biological evaluation
- Myeloid and lymphoid malignancies
- Secondary immune neutropenia
- Large Granular Leukemia
- Primary immune deficiency
- HIV and HCV infections
Chronic Primary Neutropenia
Evaluate neutrophil antibodies
- Early onset
- Cyclic neutropenia
- Familial history of neutropenia
or myeloid malignancies
- Lymphoedema, warts,
pulmonary disease,
immunodeficiency or
monocytopenia
Suspect
&
Evaluate
Genetic disease
Chronic
Secondary
Neutropenia
Yes
No
Yes
No
Blood 2015
Ethnic neutropenia
- Moderate
- Ancient
- No symptoms
- Ethnical origin
=> surveillance
NCSIA vs neutropénie ethnique ?
≠ neutropénie constitutionnelle
= variation de la normale
Asymptomatique
Pas de test biologique ayant VPP/VPN suffisante
Faisceau d’argument (origine ethnique, NFS antérieures, recul)
Afrique sub-saharienne / Amérique du Nord / Antilles
Moyen Orient
Et probablement toutes les ethnies dans une moindre mesure …
Parfois très profondes (< 0,5 G/L)
NCSIA vs neutropénie ethnique ?
Etude US (<1.5 G/L) : - « Whites » : 0.8 % - « Afro-americans » : 4.5 % - « Mexican- americans »: 0.38 %
Etude Moyen Orient - « Arabs » : 10.7 % - « Arabs bedouins » : 20 % - Ouganda : 30 %
Hsieh, Ann Intern Med 2007
Denic, BMC Blood disorders , 2009
NCSIA vs hémopathie myéloïde
Etude Danoise (NFS prescrites par les MG sur 20% population - > 378 000
pts sur 5,5 ans), prévalence neutropénie
- Aigue < 1.8 G/L : 2 %
- Aigue <1.5 G/L : 0,9 %
- Chroniques < 1.8 G/L : 0,12 %
- Chroniques < 1.5 G/L : 0,06 %
Andersen, J Intern Med 2016
NCSIA vs hémopathie myéloïde
Etude Danoise (NFS prescrites par les MG sur 20% population - > 378 000
pts sur 5,5 ans), prévalence neutropénie
- Aigue < 1.8 G/L : 2 %
- Aigue <1.5 G/L : 0,9 %
- Chroniques < 1.8 G/L : 0,12 %
- Chroniques < 1.5 G/L : 0,06 %
-> Survenue pathologie dans les 4 ans ? maladies auto immunes*, infections virales, hémopathies
Andersen, J Intern Med 2016
Infections virales : VIH +++, HCV, HBV
Hémopathies : LAM, MDS
NCSIA vs hémopathie myéloïde
Etude Danoise (NFS prescrites par les MG sur 20% population - > 378 000
pts sur 5,5 ans), prévalence neutropénie
- Aigue < 1.8 G/L : 2 %
- Aigue <1.5 G/L : 0,9 %
- Chroniques < 1.8 G/L : 0,12 %
- Chroniques < 1.5 G/L : 0,06 %
-> Survenue pathologie dans les 4 ans ? maladies auto immunes*, infections virales, hémopathies
Andersen, J Intern Med 2016
Infections virales : VIH +++, HCV, HBV
Hémopathies : LAM, MDS
Neutropénie aigue ou chronique
- Risque de mortalité accrue dans les 4 ans
- Quelque soit la sévérité
- Augmente avec la sévérité
- PNN < 0.5 G/L = IC 40 % hémopathie 4 ans
NCSIA vs Neutropénies secondaires
Sicre Blood 2015
Etude rétrospective (cohorte prospective) : 108 pts PNN < 0,5 ou < 1 G/L + spt FU médian 8.3 ans
Table S2: Causes of exclusion from the study
Patients excluded
All patients 123
Gender male 37 /123 (30%)
Exclusion reason
Pancytopenia 3 /123 (2.4%)
ANC > 0.5 109/L 39 /123 (31.7%)
Transitory neutropenia 1 (<1%)
Follow up < 1year or lack of data 22 /123 (17.9%)
Congenital neutropenia 14 /123 (11.4%)
Suspected* 11
Confirmed 3
MonoMAC syndrome 1
ELANE mutation 1
G6PC3 mutation 1
Large granular leukemia 14 /123 (11.4%)
Felty's syndrome 4 /123 (3.25%)
Sjogren's syndrome 11 (8.9%)
Systemic lupus erythematosus 2 (1.6%)
Myelodysplastic syndrome 8 (6.5%)
Others 5 (4.0%)
Others hematological malignancies 1
HCV infection 1
HIV infection 1
Drug toxicity 2
Footnotes: in the absence of genetic confirmation, congenital neutropenia was considered when the first
manifestations were reported during infancy (5 patients) and/or when an associated congenital organ dysfunction
(3 patients) was present, when neutropenia was diagnosed in a first degree relative (1 patient) or when an
immune deficiency was associated (2 patients).
Exclus 123 patients
NCSIA vs Neutropénies secondaires
• Neutropénies médicamenteuses
• Neutropénies AI associées aux hémopathies lymphoïdes
• LLC
• LNH B bas grade
Schivdel, Ann Hematol 2013
Visco, Am J Hematol 2014
Rare Anti PNN ou chronic T cell lymphocytosis (Rustagi, BJH 1987) Complications infectieuses, pronostique sévère (EVANS)
NCSIA vs Neutropénies secondaires
• Lupus érythémateux disséminé
• Syndrome de Goujerot Sjogren
• Thyroïdite auto-immunes
• ANCA
Lupus Erythémateux Disséminé
• 3 études de prévalence
• Neutropénie modérée ++
• 25-40 % ( def PNN < 1.5-2.5 G/L)
• Neutropénie sévère < 4%
• Associée à l’évolutivité de la maladie
• Association aux infections controversée (TTT IS)
• Réponse GCSF
Linda, Sem Arth Rheum 2014
Syndrome de Goujerot Sjogren
PNN < 1 G/L (10%) : 50% hospitalisation pour infection (vs 9%, p=0.002) Facteur prédictif majeur LNH
NCSIA vs Neutropénies secondaires • Thyroïdites auto-immunes
• Association fréquente ++
• Chronologie variable
• Evolution dissociée
• Lien de causalité ?
• Cases reports ++
• Marqueur d’auto-immunité à rechercher +++
• ANCA
• Neutropénie sévère
• Mécanisme humoral ++
• Association à des vascularites à ANCA rares
• Formes le + souvent cutanées
Antineutrophil Cytoplasmic Antibodies, Autoimmune
Neutropenia, and Vasculitis
Peter C. Grayson, MD1, J. Mark Sloan, MD2, John L. Niles, MD3, Paul A. Monach, MD, PhD1,
and Peter A. Merkel, MD, MPH1
1Vasculitis Center, Section of Rheumatology and the Clinical Epidemiology Unit, Department of
Medicine, Boston University Medical Center, Boston, MA
2Section of Hematology/Oncology, Department of Medicine, Boston University Medical Center,
Boston, MA
3Renal Unit, Department of Medicine, Massachusetts General Hospital, Boston, MA
Abstract
Objectives—Reports of an association between antineutrophil cytoplasmic antibodies (ANCA)
and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-
associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the
association between ANCA, neutropenia, and vasculitis is reviewed.
Methods—Longitudinal clinical assessments and laboratory findings are described in a patient
with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010.
A PubMed database search of the medical literature was performed for papers published from
1960 through October 2010 to identify all reported cases of ANCA and neutropenia.
Results—A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-
proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA
with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil
membrane antibodies were detected during an acute neutropenic phase and were not detectable in
a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An
association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the
context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without
underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other
antibodies were common; however, vasculitis was uncommon and when it occurred was usually
limited to the skin and in cases of underlying toxin exposure.
Conclusions—ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely
occurs in association with ANCA and neutropenia. The interaction between neutrophils and
ANCA may provide insight into understanding both autoimmune neutropenia and AAV.
© 2011 Elsevier Inc. All rights reserved
Corresponding Author: Peter C. Grayson MD Vasculitis Center, Section of Rheumatology Boston University School of Medicine 72East Concord Street Boston, MA 02118 [email protected] phone: 617-414-2508 Fax: 617-414-2510. Reprint Requests: Peter A.Merkel, MD, MPH Vasculitis Center, Section of Rheumatology Boston University School of Medicine 72 East Concord StreetBoston, MA 02118.
Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our
customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of
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COMPETING INTERESTS None
NIH Public AccessAuthor ManuscriptSemin Arthritis Rheum. Author manuscript; available in PMC 2012 December 1.
Published in final edited form as:
Semin Arthritis Rheum. 2011 December ; 41(3): 424–433. doi:10.1016/j.semarthrit.2011.02.003.
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Grayson, Semin Arthritis and Rheum 2011
Original article
Antineutrophil cytoplasmic antibody-associated neutropenia
Paul Coppoa,1, David Gheza,1, Vincent Fuentesb, Djaouida Bengoufac, Eric Oksenhendlera,Bruno Triboutd, Jean-Pierre Clauvela, Kaiss Lassouedb,*
aService d’Immuno-Hematologie, Hopital Saint-Louis, ParisbService d’Immunologie, CHU d’Amiens, France
cLaboratoire d’Immunopathologie, Hopital Saint-Louis, ParisdService de Pathologie Vasculaire, CHU d’Amiens, France
Received 14 January 2004; received in revised form 1 July 2004; accepted 31 August 2004
Abstract
Background: Antineutrophil cytoplasmic antibodies (ANCA) can be associated with various disorders. However, their association with
neutropenia has never been reported.
Methods: Nine patients with chronic unexplained neutropenia and ANCA were studied. Clinical charts were extensively analyzed and all
patients underwent hematological and immunological investigations.
Results: All patients (6 women and 3 men) were Caucasian and had a mean age of 49 years (range 16–67 years). All presented with a
neutropenia below 1.5 109/L for more than 6 months. The neutropenia was b0.5 109/L in six cases and moderate in three. There was no
evidence of toxic- or drug-related neutropenia or of ahematological malignancy. Autoimmune anemiaand/or thrombocytopenia werepresent
in five patients. ANCA, with various specificities, were present in all patients. ANCA were associated with various other autoantibodies in
eight patients, including antisurface-neutrophil antibodies in three cases. Four of the six patients with severe neutropenia experienced
infections. Five patients were treated with hematopoietic growth factors, steroids, intravenous immunoglobulins, splenectomy, methotrexate
and/or cyclophosphamide, allowing the neutrophil count to be restored transiently or permanently.
Conclusions: A subset of patients with neutropenia of possible autoimmune origin may develop ANCA. Their detection would provide
strong evidence of an autoimmune mechanism. Neutropenia should be added to the list of ANCA-associated diseases.
D 2004 Elsevier B.V. All rights reserved.
Keywords: Neutropenia; Antineutrophil cytoplasmic antibodies; Autoimmunity
1. Introduction
Antineutrophil cytoplasmic antibodies (ANCA) are
autoantibodies that are directed against different neutrophil
antigens. When detected by immunofluorescence on etha-
nol-fixed human neutrophils, ANCA usually displays three
major patterns: cytoplasmic (cANCA), perinuclear
(pANCA), and atypical (xANCA). The main targets of
ANCA are either myeloperoxydase (MPO) or proteinase 3
(PR3) [1–4], but they may also be directed against
lactoferrin, elastase, cathepsin, lysozyme [5], bactericidal
permeability increasing protein (BPI), and azurocidin [6].
ANCA are observed in a large spectrum of diseases [7–19].
Their specificity can make them a helpful tool in the
diagnosis of primary systemic vasculitides [20]. Anti-PR3
antibodies (Abs) are strongly associated with Wegener’s
diseaseand, to a lesser extent, with microscopic polyarteritis
and necrotizing crescentic glomerulonephritis [7–9]. Anti-
MPO antibodies are often associated with systemic vascu-
litis but can also be found in various autoimmune disorders
and connective tissuediseaseswithout evidenceof vasculitis
[10,11,13,14,16–18]. Other specificity is not considered to
0953-6205/$ - see front matter D 2004 Elsevier B.V. All rights reserved.
doi:10.1016/j.ejim.2004.08.009
* Corresponding author. Serviced’Immunologie, Faculte deMedecine,
3 rue des Louvels, F-80036 Amiens Cedex 1, France. Tel.: +33 3 22 82 79
06; fax: +33 3 22 82 79 07.
E-mail address: [email protected] (K. Lassoued).1 Contributed equally to this work.
European Journal of Internal Medicine 15 (2004) 451–459
www.elsevier.com/locate/ejim
Coppo Eur J Int Med 2011
NCSIA vs Lympho-proliférations LGL
• Caractérisation phénotypique (CMF) :
• LGL T : TCRαβ, CD3+ CD8+ CD4- CD57+.
• LGL NK : CD3-CD8+CD57+CD16+
• Clone T : LGL T, syndrome de Felty
OMS 2008
leucémies à LGL T
lymphoproliférations chroniques NK
Excès de LGL, > 6 mois, sans autre étiologie
Critères diagnostiques n’incluent ni clonalité, ni seuil
NCSIA vs Lympho-proliférations LGL
Entité clinique :
• 55 – 60 ans
• Splénomégalie, manifestations articulaires (PR atypique)
• Neutropénie (asymptomatique), érythroblastopénie
• Auto-immunité biologique : FR/hypergamma
• Evolution chronique, indolente (mais hétérogène)
Attention aux formes secondaires : • Hémopathies myéloïdes (SMD) • Hémopathies lymphoïdes • Infections virales chroniques • Asplénie
NCSIA vs Lympho-proliférations LGL
Dinmohamed AG
1ère étude prévalence non biaisée Registre des cancer aux Pays Bas = Exhaustivité > 95%
NCSIA vs Lympho-proliférations LGL
Dinmohamed AG
1ère étude prévalence non biaisée Registre des cancer aux Pays Bas = Exhaustivité > 95%
Equivalent à prévalence de 1600 cas en France sur 20 ans
NCSIA vs Déficit immunitaire
• DICV • ALPS • CID
• IPEX / CD25 deficiency • Neutropénies sévères congénitales • Syndrome MonoMAC
Euroclass trial, Wehr Blood 2008
NCSIA DÉMARCHE DIAGNOSTIQUE PNN < 1.5 G/L isolated
• Familial history
• Clinical evaluation
Clinical & biological evaluation
- Myeloid and lymphoid malignancies
- Secondary immune neutropenia
- Large Granular Leukemia
- Primary immune deficiency
- HIV and HCV infections
Chronic Primary Neutropenia
Evaluate neutrophil antibodies
- Early onset
- Cyclic neutropenia
- Familial history of neutropenia
or myeloid malignancies
- Lymphoedema, warts,
pulmonary disease,
immunodeficiency or
monocytopenia
Suspect
&
Evaluate
Genetic disease
Chronic
Secondary
Neutropenia
Yes
No
Yes
No
Blood 2015
Ethnic neutropenia
- Moderate
- Ancient
- No symptoms
- Ethnical origin
=> surveillance
Bilan proposé sauf neutropénie ethnique
Frottis
Myélogramme + caryo, pas systématique mais toujours - si < 0,5 G/L (1 G/L pour certains) - Si infection(s) - Si autre anomalie même minime (thrombopénie, macrocytose…)
Phénotype lymphocytaire avec recherche de LGL T & NK et hémopathie
lymphoïde B EPP : hypo ou hypergamma, pic
Sérologies hépatites B, C et VIH
FAN, FR, Anti SSA/SSB, ANCA
BOM pour certains en cas de neutropénie profonde et myélo NC
Neutropénie Idiopathique Chronique de l’Adulte
References Number, type of patients / type of neutropenia
Severity of neutropenia
Ratio F/M Median age (Years)
Clinical auto immunity
AIN explored? AIN Outcome and follow up
Kyle, 19681 & Kyle, 198013 Unicentric
15 (primary, adults)
ANC 0.5-1G/L (n=2) and <0.5 G/L (n=12)
14/1 41 Yes Yes 13% No severe infection Follow up: 15 y
Van der Veen, 198514 Multicentric
49 (primary, adults and children)
ANC 1-1.8 G/L (n=19), 0.5-1 G/L (n=15), <0.5 G/L
(n=15)
31/18 Na, 12 < 20 years including 9 < 10
years
Yes, no autoimmunity
Yes 15% Bacterial infection in 26%
Hartman, 19945 Unicentric
148 (primary & secondary)
ANC< 1.5 G/L Na Na Na Yes 36% Na, biological studies only
Bux, 19917 Multicentric
101 (primary, mainly children) median age 12
months
ANC <1.5 G/L, median ANC 0.250
G/L
Na 12 months No Yes 96% Few severe bacterial infections in primary AIN
Logue, 19914 Multicentric
71 (adults)
ANC <1.5 G/L 51/20 46.2 Na Yes 32% Recurrent infections in 14% (FU unknown).
Dale, 200311 & Dale, 200612 Multicentric
224 (adults) ANC < 0.5 G/L 171/53 Na No Na Na Na
Dancey, 198015 Unicentric
10 (adults)
ANC < 0.5 G/L (n=3), ANC 0.5-2
G/L (n=7)
5/5 49 No No Na No infection and no aphtous stomatitis with a median follow up of 2.2y. Follow up: 2.2 y
Fattizzo, Eur Jour of Intern Med 2015 Multicentric Propsective
76 (primary adults, prospective)
ANC < 1.5 G/L, ANC < 0.5 G/L (n=9)
Median 1.27 G/L
51/33 56 (15-86)
Na Yes 35% Grade 2 infections only in 25% 12 patients : SMD, LGL, HCL FU 5 years
Papadaki, 199916 Unicentric
32 ANC < 0.5 G/L (n=1) ANC 1 – 1.5 G/L
(n=31)
28/4 60 No Yes No Follow up: 7.5 y
Neutropénie Idiopathique Chronique de l’Adulte
Etude rétrospective (cohorte prospective) : 108 pts PNN < 0,5 ou < 1 G/L + spt FU médian 8.3 ans
Sicre Blood 2015
Neutropénie Idiopathique Chronique de l’Adulte
Sicre Blood 2015
Etude rétrospective (cohorte prospective) : 108 pts PNN < 0,5 ou < 1 G/L + spt FU médian 8.3 ans
Neutropénie Idiopathique Chronique de l’Adulte
Blood cell count
Sicre Blood 2015
Caractéristiques hématologiques
Neutropénie Idiopathique Chronique de l’Adulte
Blood cell count
Sicre Blood 2015
Caractéristiques hématologiques
Neutropénie Idiopathique Chronique de l’Adulte
Caractéristiques immunologiques
Gammaglobulins
Neutropénie Idiopathique Chronique de l’Adulte
Manifestations associées
Neutropénie Idiopathique Chronique de l’Adulte
Manifestations associées
Table S4: Severe bacterial infections
Severe bacterial infections
Number of patients with severe bacterial infections
At least 1 27/108 (24.5%)
1 15/108 (13.9%)
2 7/108 (6.5%)
3 5/108 (4.6%)
Total number of severe bacterial infections 45
Abscess/cellulitis 24 (53%)
Pneumonia 7 (15%)
Tonsil phlegmon's 4 (9%)
Pyelonephritis 4 (9%)
Peritonitis 2
Adenitis 2
Osteitis 1
Arthritis 1
Septic shock 2
Intensive care unit admission 3
Table S4: Severe bacterial infections
Severe bacterial infections
Number of patients with severe bacterial infections
At least 1 27/108 (24.5%)
1 15/108 (13.9%)
2 7/108 (6.5%)
3 5/108 (4.6%)
Total number of severe bacterial infections 45
Abscess/cellulitis 24 (53%)
Pneumonia 7 (15%)
Tonsil phlegmon's 4 (9%)
Pyelonephritis 4 (9%)
Peritonitis 2
Adenitis 2
Osteitis 1
Arthritis 1
Septic shock 2
Intensive care unit admission 3
IC 3.7 % 1 y 21 % 5 & 10 y 39 % 20 y
Neutropénie Idiopathique Chronique de l’Adulte
Manifestations associées
Neutropénie Idiopathique Chronique de l’Adulte
Traitements
Réponse 40%
Médiane 3 mois
Neutropénie Idiopathique Chronique de l’Adulte
Traitements
Evaluation difficile car hétérogénéité des indications - Echec (réfractaire = 4%) - Intolérance (céphalées, douleurs osseuses : fréquent -> baisser la dose) - Epargne du GCSF (peur de l’hémopathie secondaire)
Petites séries le plus souvent de réponses favorables… - Endoxan - Methotrexate - Ciclosporine - Campath, Rituximab - Corticoïdes : réponse fréquente mais cortico-dépendance forte dose - Splénectomie : échecs
Formes réfractaire au GCSF (> 15 jours, 5 à 10 μg/kg/j)
- 4 patientes consécutives - neutropénies très sévères, compliquées infections sévères - idiopathiques 3 / AI 1 - 3 sur 4 réponse rapide (7 à 15 jours), complète au Néoral
50% de réponse
Neutropénie Idiopathique Chronique de l’Adulte
Traitements
Neutropénie Idiopathique Chronique de l’Adulte
Risque leucémique
Cohorte française : Aucune évolution vers LAM / SMD
5 cas rapportés dans la littérature - 1 seul avait reçu du GCSF - 2 : histoire familiale d’hémopathie myéloïde (monoMAC,
SBDS, SMD familial ?)
Neutropénie Idiopathique Chronique de l’Adulte
Neutropénie chronique acquises sévère ou symptomatique :
o BOM si myélogramme de richesse diminuée
o Recherche d’anticorps anti-granuleux
o Bilan de thyroïdite auto immune
o Clone T (recherche)
o Adultes jeunes, pas de NFS antérieure normale,
monocytopenie ou lymphopénie : à discuter GATA2/SBDS
Risque leucémique
www.marih.fr - [email protected]
Centres de référence maladies rares
Associations de patients
Sociétés savantes