Full Report

Format

Full Report
Summary (Text)
Summary (XML)

Send to:

Choose Destination

File
Clipboard
Collections

Splenic xanthomatosis

MedGen UID:: 1842145
•Concept ID:: C5826534
•: Disease or Syndrome

Synonym:	Spleen xanthomas

HPO:	HP:0034623

Definition

Lipid deposits localized within the spleen. Xanthomas are benign but are often an important visible sign of systemic conditions such as familial hypercholesterolemia. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSplenic xanthomatosis

Phenotypic abnormality
- Abnormality of the immune system
  - Abnormality of the lymphatic system
    - Abnormality of the spleen
      - Abnormal spleen morphology
        Splenic xanthomatosis

Recent clinical studies

Etiology

Expression of stabilin-1 in M2 macrophages in human granulomatous disease and melanocytic lesions.

Schönhaar K, Schledzewski K, Michel J, Dollt C, Gkaniatsou C, Géraud C, Kzhyshkowska J, Goerdt S, Schmieder A
Int J Clin Exp Pathol 2014;7(4):1625-34. Epub 2014 Mar 15 PMID: 24817959 Free PMC Article

Long-term kinetics of serum and xanthoma cholesterol radioactivity in patients with hypercholesterolemia.

Samuel P, Perl W, Holtzman CM, Rochman ND, Lieberman S
J Clin Invest 1972 Feb;51(2):266-78. doi: 10.1172/JCI106811. PMID: 5009114 Free PMC Article

See all (2)

Diagnosis

A case of progressive xanthogranulomatous pancreatitis with splenic abscess.

Nagata K, Nakamura K, Iida T, Iwasaki J, Ito R, Asai S, Ishihara M, Hata T, Itami A, Kyogoku T
Clin J Gastroenterol 2024 Jun;17(3):580-586. Epub 2024 Mar 8 doi: 10.1007/s12328-024-01930-1. PMID: 38457070

Pseudoxanthomatous or xanthelasmoid mastocytosis: Reporting a rare entity.

Sinha P, Sandhu S, Sen A, Sood A
Indian J Dermatol Venereol Leprol 2020 Jul-Aug;86(4):382-385. doi: 10.4103/ijdvl.IJDVL_633_17. PMID: 30785120

Necrobiotic xanthogranuloma with cardiac involvement.

Umbert I, Winkelmann RK
Br J Dermatol 1995 Sep;133(3):438-43. doi: 10.1111/j.1365-2133.1995.tb02674.x. PMID: 8547001

Wolman's disease: report of two new cases, with a review of the literature.

Raafat F, Hashemian MP, Abrishami MA
Am J Clin Pathol 1973 Apr;59(4):490-7. doi: 10.1093/ajcp/59.4.490. PMID: 4692977

Deficiency of acid esterase activity in Wolman's disease.

Young EP, Patrick AD
Arch Dis Child 1970 Oct;45(243):664-8. doi: 10.1136/adc.45.243.664. PMID: 5477680 Free PMC Article

See all (8)

Therapy

Splenic histiocyte-rich pseudotumor following chemotherapy for non Hodgkin diffuse large B cell lymphoma.

Abdou AG, Kandil M, Eldien MS, Abdallah R
Pathologica 2016 Sep;108(3):140-143. PMID: 28195266

Necrobiotic xanthogranuloma associated with paraproteinemia and non-Hodgkin's lymphoma developing into chronic lymphocytic leukemia: the first case reported in the literature and review of the literature.

Oumeish OY, Oumeish I, Tarawneh M, Salman T, Sharaiha A
Int J Dermatol 2006 Mar;45(3):306-10. doi: 10.1111/j.1365-4632.2006.02575.x. PMID: 16533236

See all (2)

Prognosis

Necrobiotic xanthogranuloma with cardiac involvement.

Umbert I, Winkelmann RK
Br J Dermatol 1995 Sep;133(3):438-43. doi: 10.1111/j.1365-2133.1995.tb02674.x. PMID: 8547001

Portacaval shunt in two patients with homozygous type II hyperlipoproteinaemia.

Cywes S, Davies MR, Louw JH, Berger GM, Bonnici F, Joffe HS
S Afr Med J 1976 Feb 21;50(8):239-43. PMID: 176738

Long-term kinetics of serum and xanthoma cholesterol radioactivity in patients with hypercholesterolemia.

Samuel P, Perl W, Holtzman CM, Rochman ND, Lieberman S
J Clin Invest 1972 Feb;51(2):266-78. doi: 10.1172/JCI106811. PMID: 5009114 Free PMC Article

Letterer-Siwe disease. A survey and a review of the course in two subchronic cases.

Nyholm K, Reed G, Sjolin KE
Acta Pathol Microbiol Scand 1967;70(4):481-500. PMID: 5582374

See all (4)

Clinical prediction guides

The spleen in type I hyperlipoproteinemia. Histochemical, biochemical, microfluorometric and electron microscopic observations.

Ferrans VJ, Buja LM, Roberts WC, Fredrickson DS
Am J Pathol 1971 Jul;64(1):67-96. PMID: 4326635 Free PMC Article

Wolman's disease with hypolipoproteinemia and acanthocytosis: clinical and biochemical observations.

Eto Y, Kitagawa T
J Pediatr 1970 Nov;77(5):862-7. doi: 10.1016/s0022-3476(70)80248-7. PMID: 5504079

See all (2)

MedGen

National Center for Biotechnology Information

Send to:

Splenic xanthomatosis

Definition

Term Hierarchy

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Clinical resources

Consumer resources

Reviews

Related information

Recent activity