Request PDF on ResearchGate | Maladie de castleman multicentrique: A propos d'un cas | La maladie de Castleman est une affection rare, d'étiologie inconnue. Castleman disease is a group of uncommon lymphoproliferative disorders characterized by . Print/export. Create a book · Download as PDF · Printable version. Cet article est une ébauche concernant la médecine et l'immunologie. Vous pouvez partager .. Créer un livre · Télécharger comme PDF · Version imprimable.
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- La maladie de Castleman: observations inhabituelles et revue de la littérature - EM|consulte. The full text of this article is available in PDF format. Clinique - Vol. 65 - N° 2 - p. - Maladie de Castleman à expression thoracique - EM|consulte. The full text of this article is available in PDF format. tvnovellas.info 1 . Blétry O, Chomette G, Godeau P. Qui se cache derrière la maladie de Castleman multicentrique?.
This is an open access article distributed under the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. It presents with fever, systemic symptoms, generalized lymphadenopathy, and laboratory markers of inflammation. Its treatment is not standardized and its prognosis is poor. The outcome was favorable after treatment by corticosteroid, chemotherapy, and surgery. This case report underlines the diagnostic difficulties and the long interval between onset and diagnosis when diarrhea occurs first. It is characterised by peripheral lymphadenopathy with systemic symptoms and laboratory markers of inflammation. Most reported cases have been described in adult patients, with a peak of incidence in the third and fourth decade of life for localized forms and in the fourth and fifth decade of life for multicentric forms [ 1 ].
Excisional lymph node biopsy is preferred for diagnosis, particularly if the patient appears to have UCD. Immunohistochemical staining for LANA or polymerase chain reaction testing can be used to detect the presence of HHV-8 within lymph node sections.
Detection of plasma HHV-8 viral DNA is not helpful because the test is not specific 18 and will be negative in idiopathic cases. Histological confirmation therefore remains essential. Pathological subtypes CD is principally distinguished by the presence of localized or generalized lymphadenopathy, designated UCD and MCD, respectively.
CD may also be classified according to specific histological features, as well as the presence or absence of viral coinfection.
The histological pattern is usually diagnostic. Typically, there are multiple tight aggregates of follicular dendritic cells FDCs or atrophic follicles, often with radially penetrating vessels, contained within expanded circumscribed clusters of mantle zone lymphocytes.
The vascular pattern between the small lymphoid nodules is also typically prominent, and perivascular hyalinization may be a feature Figure 1. There is an association between this form of the disease, various vascular tumors, and dendritic cell proliferations, such as dendritic cell sarcomas.
Examination revealed mild bilateral cervical and axillary lymphadenopathy with no palpable organomegaly. However, myeloma was excluded when serum protein electrophoresis detected no monoclonal band. Bone marrow aspiration and trephine biopsy showed small lymphoid follicular aggregates.
Excision biopsy of an axillary node was performed and this was reported as a lymphoproliferative picture increased number of follicles containing amorphous hyaline material and some small blood vessels between simple reactive changes and frank lymphoma, suggesting Castleman's disease.
He was referred to a haematologist and was commenced on steroid therapy which successfully induced disease remission and symptomatic relief. Discussion Castleman's disease CD is lymphoproliferative disorder which is histologically characterised by angiofollicular lymph-node hypertrophy [ 3 ].
This case report, together with a review of medical literature in existence, attempts to provide new insight into this rather rare and relatively benign disorder which, though mimicking lymphoma clinically, varies from the latter histologically, prognostically and in its treatment options.
Localised CD is, by definition, localised to one site. It features lymphoid hyperplasia associated with excessive angiogenesis [ 1 ].
Histological diagnosis requires lymph-node biopsy. Colon biopsies showed a collagenous colitis rich in eosinophils Figure 2 A. Gastric biopsies revealed chronic inflammation and glandular atrophia with intestinal metaplasia of the fundus suggestive of an atrophic gastritis Figure 2 B.
Prostatic biopsies showed mild chronic inflammation without neoplasia Figure 2 C. Liver biopsy showed mild sinusoidal distension, unspecific portal and lobular inflammation, and rare epithelioid granulomas without necrosis Figures 2 D,E.
Finally, the patient underwent a bronchial biopsy showing unspecific chronic inflammation without granuloma, which was not suspicious for sarcoidosis Figure 2 F. A Hematoxylin and eosin stain. B Hematoxylin and eosin stain.
C Hematoxylin and eosin stain. D Hematoxylin and eosin stain.
F Hematoxylin and eosin stain. Treatment Course and Outcome The best management of iMCD disease remains poorly defined with only one randomized clinical trial published in on siltuximab compared to best supportive care Thus, this case required multidisciplinary discussion and consensus in when the diagnosed was made and judgment was based on available data with tocilizumab 13 and rituximab for the treatment of HHVassociated MCD The rational to use methylprednisolone and tocilizumab was to rapidly stop ILmediated inflammation as induction regimen, whereas rituximab was used as maintenance treatment.
Corticosteroids were rapidly weaned off because of poor control of the diabetes, and the patient was hemodialyzed for end-stage renal disease. In the following 5 years, the patient relapsed twice, each time about 1 year after rituximab was discontinued Figure 3 A.
Auto-antibodies disappeared over the years and hypogammaglobulinemia developed, probably due to repeated rituximab treatments. Follow-up bone marrow biopsies were not performed in this case. High values of IL-6 early after tocilizumab were excluded. Most of the reported cases were Japanese patients. To analyze the role of ethnicity, we have reviewed the literature for all known Caucasian cases and have identified eight including the present one. When analyzing major and minor criteria recently established by Masaki et al.
In comparison with the Japanese cohort, of 18 patients we find Caucasian TAFRO cases presented more frequently with kidney failure and auto-immune antibodies, although these results need to be interpreted with caution due to the low number of patients Table 1. As to IgG4-related disease primary reported in Japan 22 , TAFRO syndrome is a rare entity affecting patients with different ethnic background without major differences in the clinicopathological presentation.
Recently, the international working group for the Castleman Disease Collaborative Network established the first-ever diagnostic criteria for HHVnegative-iMCD analyzing patients with iMCD patients from literature, 37 cases submitted by the working group members, and 79 from randomized control study and more specifically the histopathological features of 85 lymph nodes with presumptive diagnosis of MCD.