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Journal of Clinical Case Reports

ISSN: 2165-7920

Open Access

Case Report - (2021) Volume 14, Issue 1

Maroua Melliti*, Malek Mansour, Amel Kacem, Ines Bedoui and Ridha Mrissa
*Correspondence: Maroua Melliti, Department of Medicine, Military Hospital of Instruction of Tunis, Tunisia, Email:
Department of Medicine, Military Hospital of Instruction of Tunis, Tunisia

Received: 11-May-2021 Published: 31-May-2021
Citation: Maroua Melliti, Malek Mansour, Amel Kacem, and Ines Bedoui, et al. “Association of Multiple Sclerosis and Castleman Disease: A Case Report and Literature Review.” Clin Case Rep 11 (2021): 1443.
Copyright: © 2021 Melliti M, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Castleman Disease (CD) is an uncommon lymphoid hyperplasia occurring in the mediastinal lymph nodes, and less frequently in the neck lymph nodes. Several factors, likewise Multiple Sclerosis (MS), are reported to be involved in the mechanism of each of the diseases.

Keywords

Effectiveness; Role play; Knowledge; Personal hygiene; School going children

Introduction

We report the case of a patient with CD and MS and we review variants of CD, possible triggering factors and other association cases. We describe the case of a 27-year-old man with no family or personal history, presented with progressive gait disorder which had developed within six month with no extraneurologic symptoms such as asthenia, fever or night sweats.

Case Report

Neurologic examination revealed a static and cinetic cerebellar syndrome with posterior cord syndrome. Magnetic Resonance Imaging (MRI) revealed multiple hyper intense T2 and FLAIR lesions in both cerebral periventricular and juxtacortical, some with confluent characteristics (plaque aspect) (Figure 1), and medullar cord with no active lesion in contrast-enhanced T-weighted images, with a parapharayngal isointense T1 and hyperintense T2 mass (Figure 2). Histologic investigation was undergone after excisional biopsy revealing lymph node hyperplasia and follicles with hyalinized foci. Chest and abdomen CT scan did not show mediastinal or abdominal lymphadenopathies. Serological studies for HIV, Hepatite C Virus (HCV) and Human Herpes virus (HHV)-8 were negative with final diagnosis of Unicentric Castleman Disease (UCD) as extensive workup studies failed to show other localization outside the excised lymph node. Immunologic assessments as well as labial gland biopsy were negative. Cerebrospinal Fluid (CSF) immune electrophoresis revealed the presence of oligoclonal bands as well as increased concentration of CSF IgG. Diagnosis of Multiple sclerosis and UCD was established. He was treated by beta interferon with favourable outcome.

Figure

Figure 1. Brain MRI axial FLAIR (Arrow A) and T2 (Arrows C and D), sagittal T2- weighted images (Arrow B) show hyper intense periventricular and juxtacortical lesions suggestive for MS.

Figure

Figure 2. Sagittal MRI images shows isointense T1 (Arrow A) and hyperintense T2- weighted images (Arrow B) parapharayngal mass with central linear hypointense septate suggestive for lamellar fibrosis.

Discussion

Castleman disease was first described by Benjamin Castleman in 1954 as an unusual lymphoid hyperplasia occuring predominantly in mediastinium and neck of young to middle age patients [1]. According to histopathological features and clinical data, three variants are universally accepted [2]: a Uni Centric Variant (UCD) with Hyaline-Vascular (HV) features, a UCD Plasma Cell (PC) type with sheets of polytypic PC in the inter follicular area and a multicentric-type CD (MCD) predominantly showing histological features of the PC type. Similarly to Multiple Sclerosis (MS), the pathogenesis of CD is still a matter of debate. Both environmental and genetic factors are involved in the etiology of MS. A role for some viral infections, such as HHV-8 has been proposed in MS which, associated with HIV infection, has a fundamental role in Multicentric Castleman disease [3,4]. In contrast, the majority of patients with HV UCD patients do not have laboratory abnormalities which is the case of our patient [5]. Reported associated diseases to UCD encompass paraneoplastic pemphigus and follicular dendritic cell sarcoma. The association with Hodgkin Disease (HD) is commonly described with PC variant histology and an interfollicular variant of HD [5].

Association of CD and MS was reported in rare cases [6,7]. UCD variant was described in a 54-year-old female suffering from MS since the age of 44 and treated with beta interferon, developing a lymph node hyaline-vascular type CD relapsing in the skin after 24 months. The second case was of a 57-year-old female who was shown to have an episode with fever, sweating, lumbalgia and purpuric rash of the right leg, which was diagnosed with MCD, and who then developed gait and speaking disorder within one month and was diagnosed with MS after cerebral and medullar MRI [7]. For our patient, MS was inaugurated with gait disorder however UCD was totally asymptomatic. UCD typically presents as a solitary mass with a progressive enlargement and indolent course. It can be asymptomatic or may have symptoms due to compression of adjascent structures. It occurs equally for men and women and the median age of presentation is 30 years and 34 years old [5]. Approximately 75% to 90% of patients with UCD have HV histologic subtype whereas 10% to 25% patients have variants. Imaging studies typically showed a solitary lesion, as the case of our patient, which may mimic a lymphoma. There is no reliable diagnostic method and its defenetive diagnosis is based on histopathology report [5]. CD was also described in three cases of alemtuzumab-treated patients suffering from active remitting-relapsing MS, as potentially lifethreatening secondary autoimmune event.

The diagnosis of idiopathic MCD (iMCD) and TAFRO (Thrombocytopenia, Anasarca, MyeloFibrosis, Renal failure, Organomegaly) subtype (typical histopathologic findings, HHV-8 negative immunohistochemistry, thrombocytopenia, fever, organomegaly, absence of hypergammaglobulinemia (0.8 g/d)) was established after histopathologic studies of lymph node revealing hyperplasia, germinal centre regression, plasma cell expansion and hyalinised vessels [8]. The disease onset has been reported at 28 to 41 months after alemtuzumab initiation [9-12]. As association with different malignancies can occur before, concurrent with or after iMCD (with an incidence of up to 19%), a thorough clinical follow up is required especially beyond 48 months after the last alemtuzumab infusion [13]. However, patients with UCD variant have excellent prognosis which confers 10-year overall survival rates of more than 95% [14] especially after complete resection of the mass. If complete resection is not possible, radiation therapy can be considered with an overall survival rate of 82% at 20 months with limited data on long-term response rate [15]. Systemic therapeutic options for MCD, such as chemotherapy, rituximab or anti-Interleukin-6 (IL-6) therapy can be considered for patients with UCD who cannot be treated with surgery or radiotherapy or for those who fail to respond to such treatment. The peculiarity of the present case bases itself on the association of UCD discovered incidentally in treatment free MS-patient which is to our best of knowledge, the first reported case of such a possible association.

Conclusion

Multiple sclerosis is a myelodegenerative disease sustained by production of anti-myelin antibodies. The deregulated immune system might have favoured the development of CD. Although many environmental factors are involved, the precise mechanism which triggers both diseases is still inconclusive. Future studies with larger sample sizes and evaluation of immune status of patients are warranted.

References

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