Erdheim-Chester disease and Schnitzler syndrome: so near, and yet so far

Kód výsledku v IS VaVaI

RIV/00216224:14110/13:00070504 - isvavai.cz

Výsledek na webu

http://dx.doi.org/10.1007/s12185-013-1437-2

DOI - Digital Object Identifier

10.1007/s12185-013-1437-2

Jazyk výsledku

angličtina

Název v původním jazyce

Erdheim-Chester disease and Schnitzler syndrome: so near, and yet so far

Popis výsledku v původním jazyce

A 56-year-old male presented with diabetes insipidus, noninfectious fever, night sweats, and pain in both lower legs and pelvis. Developing gradually over a period of 9 years, these symptoms were accompanied by increased acute phase markers (C-reactive protein = 63 mg/L, erythrocyte sedimentation rate = 52 mm/h and 72 mm/2 h, fibrinogen = 6.5 g/L). Technetium-99m pyrophosphate bone scintigraphy showed abnormal tracer uptake predominantly in the long bones of the lower extremities, with additional hot spots in the skull, right shoulder, left humerus, and both radial bones (Fig. 1). Positron emission tomography (PET) identified a similar pattern of 18F-fluorodeoxyglucose uptake correlating with osteosclerotic lesions on conventional radiography (X-ray) and computed tomography(CT). Subsequently, histological findings of CD68- and S100-positive, CD1a-negative foamy histiocytes from a bone biopsy were compatible with Erdheim?Chester disease.

Název v anglickém jazyce

Erdheim-Chester disease and Schnitzler syndrome: so near, and yet so far

Popis výsledku anglicky

A 56-year-old male presented with diabetes insipidus, noninfectious fever, night sweats, and pain in both lower legs and pelvis. Developing gradually over a period of 9 years, these symptoms were accompanied by increased acute phase markers (C-reactive protein = 63 mg/L, erythrocyte sedimentation rate = 52 mm/h and 72 mm/2 h, fibrinogen = 6.5 g/L). Technetium-99m pyrophosphate bone scintigraphy showed abnormal tracer uptake predominantly in the long bones of the lower extremities, with additional hot spots in the skull, right shoulder, left humerus, and both radial bones (Fig. 1). Positron emission tomography (PET) identified a similar pattern of 18F-fluorodeoxyglucose uptake correlating with osteosclerotic lesions on conventional radiography (X-ray) and computed tomography(CT). Subsequently, histological findings of CD68- and S100-positive, CD1a-negative foamy histiocytes from a bone biopsy were compatible with Erdheim?Chester disease.

Druh

O - Ostatní výsledky

CEP obor

FD - Onkologie a hematologie

OECD FORD obor

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Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2013

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů