Maladies systémiques au cours des syndromes myélodysplasiques

Hebbar, Mohamed; Hebbar-Savéan, K; Fenaux, Pierre

doi:10.1016/0248-8663(96)80810-1

Cited by 18 publications

(2 citation statements)

References 47 publications

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“…To further support our theory that our patient suffered from RPC, it is interesting to note the known association of RPC with MDS as a paraneoplastic phenomenon (Michet et al 1986;Hebbar et al 1995). In their series, Hebbar et al reviewed the cases of patients with MDS and RPC that were reported in sufficient detail in the literature and found a number of similarities with our case.…”

Section: Discussionmentioning

confidence: 74%

Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis

Loeffler¹,

McLean²

2000

Acta Ophthalmologica Scandinavica

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ABSTRACT.Purpose: The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. Case Report/Methods: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. Results: Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. -The deafness had been due to inner ear failure, and the patient died of a cardiac failure. Conclusions: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.

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Section: Discussionmentioning

confidence: 74%

Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis

Loeffler¹,

McLean²

2000

Acta Ophthalmologica Scandinavica

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“…In terms of pathophysiology, immunological abnormalities are observed during myelodysplastic syndromes. They are related to disturbances in humoral and cellular immunity, B and T lymphocyte interaction, abnormalities of NK cells and mono-nucleated phagocytes [31]. The secretion of interleukin 2, interferon-gamma, and tumor necrosis factor alpha (hematopoietic inhibitor cytokine) is the reflection of deviations in the distribution of TH (T Helper) and TC (cytotoxic T) cells.…”

Section: Discussionmentioning

confidence: 99%

Neurological Facets of Hematological Malignancies: A Case Series

Ghachem,

Fray,

Jamoussi

et al. 2023

Recent Adv Clin Trials

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Background: Central nerve involvement is a serious complication in hemopathies. Clinical presentations are heterogeneous; it can affect the central nervous system as well as the peripheral. The pathophysiological mechanisms are multiple. Aims: To illustrate the diversity of these neurological manifestations affecting both the central and peripheral nervous systems. We study the pathophysiological mechanisms and diagnostic strategies. We discuss the therapeutic alternatives and the prognostic value of neurological damage. Methods: To illustrate the clinical and etiological polymorphism, we report a series of clinical cases of seven patients followed for malignant hemopathies and who consulted for different neurological manifestations. Results: We have seven patients followed for malignant hemopathies: Acute lymphoblastic Leukemia (ALL), Acute myeloblastic Leukemia (AML), Myelodysplasic syndrome (MDS), Chronic myeloid Leukemia (CML). Patients consult for different neurological manifestations (optic neuritis, neuropathic pain, sensory and motor deficit, myopathic syndrome. The etiological assessment identified different causes and mechanisms: leukemic medullary infiltration (myelitis) and optic nerve (optic neuritis). Acute post infectious polyradiculoneuritis in a patient undergoing chemotherapy. The neurological manifestations related to drugs were painful peripheral neuropathy and myopathic syndrome secondary to cytarabine syndrome. The fatal outcome was associated with extensive infiltration and fulminant neurovascularitis induced by MDS. Conclusion: We discuss here through our series the different epidemiological, physiopathological, clinical, evolutionary and prognostic aspects of neurological complications of hematological malignancies.

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Rezidivierende Polychondritis: ein Chamäleon unter den seltenen Erkrankungen

Schumacher

Pieringer

2017

Wien Med Wochenschr

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Relapsing polychondritis (RPC) is a rare disease with recurrent episodes of inflammation of cartilage tissue leading to fibrosis and organ damage. Despite unknown etiology, there is some evidence of a genetic predisposition. The clinical presentation is heterogeneous and an association with other autoimmune disorders such as rheumatoid arthritis or different forms of vasculitis has been described. All organ systems containing cartilage can be affected, such as ear, nose, joints, trachea, aorta, and coronary arteries. Given the broad spectrum of potential manifestations, a variety of medical specialists may be involved in the management of RPC patients. As establishing the diagnosis of RPC may be difficult, an interdisciplinary approach may be preferable. Treatment options include glucocorticoids, dapsone, disease-modifying antirheumatic drugs, and biologics. Prognosis is as heterogeneous as the clinical picture, depending on the severity of organ damage. In this paper we give an overview of the current knowledge with regard to pathogenesis, clinical picture, diagnosis, and therapy of RPC.

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Maladies systémiques au cours des syndromes myélodysplasiques

Cited by 18 publications

References 47 publications

Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis

Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis

Neurological Facets of Hematological Malignancies: A Case Series

Rezidivierende Polychondritis: ein Chamäleon unter den seltenen Erkrankungen

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