Diffuse Plane Normolipaemic Xanthomatosis in a Patient with Chronic Lymphatic Leukaemia and Monoclonal Gammopathy

Stockman, Annelies; Delanghe, Joris; Geerts, Marie-Louise; Naeyaert, Jean

doi:10.1159/000063384

Cited by 21 publications

(29 citation statements)

References 9 publications

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“…However, Touton cells have been observed in HX and NX in several cases in the literature. 20,49,57,62,[78][79][80][81][82][83][84][85] Moreover, as demonstrated here, foam cells and Touton cells share phenotypic characteristics, including the surface expression of the CD163 antigen, which would suggest a common macrophagic origin. Finally, immune complex formation resulting from antibody activity of the MIg directed against various lipoproteins appears to represent a common physiopathologic pathway leading to a lipid accumulation in the macrophagic cells.…”

Section: Discussionmentioning

confidence: 74%

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Szalat

Arnulf

Karlin

et al. 2011

Blood

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Xanthomas are a common manifestation of lipid metabolism disorders. They include hyperlipemic xanthoma, normolipemic xanthoma, and a related condition, necrobiotic xanthogranuloma (NXG). All 3 forms can be associated with monoclonal immunoglobulin (MIg). In an attempt to improve diagnosis, understanding, and treatment of this association, we retrospectively analyzed a personal series of 24 patients (2 hyperlipemic xanthoma, 11 normolipemic xanthoma, and 11 NXG) and 230 well-documented reports from the literature. With the exception of the nodules and plaques featured in NXG, the clinical presentation of xanthomatous lesions usually resembled that seen in common hyperlipidemic forms and could not be used to suspect MIg-associated xanthomas. Extracutaneous sites were not rare. The MIg was an IgG in 80% of cases. Myeloma was diagnosed in 35%. Hypocomplementemia with low C4 fraction was present in 80% of studied patients. Low C1 inhibitor serum levels were found in 53%. Cryoglobulinemia was detected in

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Section: Discussionmentioning

confidence: 74%

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Szalat

Arnulf

Karlin

et al. 2011

Blood

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“…In adults, there have been sporadic reports of the development of a variety of histiocytic disorders, including diffuse plane normolipemic xanthomatosis, [16,17] and necrotic [18] and eruptive, disseminated [19] xanthogranulomas that have preceded or arisen concurrently with a variety of hematologic malignancies such as myeloma and chronic lymphocytic leukemia. [16,18,19] In one case, the patient developed diffuse plane normolipemic xanthomatosis and pancytopenia and, following bone marrow biopsy, was diagnosed with myelodysplastic syndrome and monoclonal gammopathy of undetermined significance.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Xanthogranulomas, Hepatosplenomegaly, Anemia, and Thrombocytopenia as Presenting Signs of Juvenile Myelomonocytic Leukemia

Cham

Siegel

Ruben

2010

American Journal of Clinical Dermatology

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The development of xanthogranulomas has been linked to hematologic malignancies in children and adults, based on a number of reports in the literature. In children, a specific association between juvenile xanthogranuloma, neurofibromatosis 1, and juvenile myelomonocytic leukemia has been described. We report a case of a 9-month-old child, without a known diagnosis of neurofibromatosis 1, who presented with hepatosplenomegaly, anemia, thrombocytopenia, and multiple cutaneous nodules, which were confirmed to be juvenile xanthogranulomas upon biopsy. A concurrent work-up showed that the child had juvenile myelomonocytic leukemia. Although cutaneous juvenile xanthogranulomas are benign lesions, in several reported cases they have been shown to herald leukemia. This association between xanthogranulomas and hematologic malignancy is poorly understood. Juvenile xanthogranulomas have a number of morphologic variants and clinical presentations that can be confused with the cutaneous lesions of Langerhans cell histiocytosis and dermatofibroma. Recognition of the broad clinicopathologic spectrum of juvenile xanthogranulomas is critical for proper diagnosis.

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“…NCXs type II have been repeatedly reported in patients with paraproteinaemia: multiple myeloma [5, 6], Waldenström’s macroglobulinaemia [7], chronic myelomonocytic and myeloid leukaemia [8], chronic lymphatic leukaemia [9, 10], benign monoclonal gammopathy [11, 12]or lymphoma [13]. Diffuse plane normolipaemic xanthomas are typically yellowish plaques involving flexures in the face (eyelids), neck and trunk areas and upper extremities in normolipaemic patients.…”

Section: Discussionmentioning

confidence: 99%

Dystrophic Xanthomatosis in Primary CD30-Positive Cutaneous T-Cell Lymphoma

et al. 2005

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The development of xanthomatous changes in lesions of primary cutaneous T-cell lymphomas (CTCL) is a rare event. It is usually observed in regressing skin tumours or plaques spontaneously or after a specific treatment. The pathogenetic mechanisms implicated in these changes are poorly understood. We are reporting 2 cases of extensive dystrophic xanthomatous changes, developing in regressing lesions of primary cutaneous CD30-positive T-cell lymphoproliferative disorders. Previously reported cases of primary CTCL developing secondary (dystrophic) xanthomata are reviewed.

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Diffuse Plane Normolipaemic Xanthomatosis in a Patient with Chronic Lymphatic Leukaemia and Monoclonal Gammopathy

Cited by 21 publications

References 9 publications

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Pathogenesis and treatment of xanthomatosis associated with monoclonal gammopathy

Cutaneous Xanthogranulomas, Hepatosplenomegaly, Anemia, and Thrombocytopenia as Presenting Signs of Juvenile Myelomonocytic Leukemia

Dystrophic Xanthomatosis in Primary CD30-Positive Cutaneous T-Cell Lymphoma

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