Crystal-Storing Histiocytosis: Report of a Case, Review of the Literature (80 Cases) and a Proposed Classification

Doğan, Snjezana; Barnes, Leon; Cruz-Vetrano, Wilhelmina P.

doi:10.1007/s12105-011-0326-3

Cited by 127 publications

(219 citation statements)

References 51 publications

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“…Inflammatory conditions such as chronic infection and autoimmune disease have been suggested as a possible risk factor for CSH. 5 Consistent with this possibility, 3 patients (including case 2 presented above) had histories of chronic lung infection, and 1 may have had an unrecognized autoimmune disease (case 1 presented above). Treatment and follow-up data were available only for 6 of the 12 patients, 5 of whom were treated with a wedge resection or lobectomy and 1 patient declined surgical treatment and was followed up with CT scans.…”

Section: Review Of Published Casesmentioning

confidence: 80%

“…A follow-up CT a year later showed interval growth of the left upper lobe nodule, which now appeared as a partially calcified 2.5-cm mass, and other bilateral nodules ranging in size between 5 mm and 2 cm were present, most of which appeared to be stable in size. A whole body PET-CT revealed high fluorodeoxyglucose uptake in the dominant left upper lobe mass (standard uptake units 20), as well as additional bilateral nodules measuring up to 2.1 cm (standard uptake units [4][5][6][7][8]. The findings were suggestive of malignancy, and a CT-guided biopsy of the left upper lobe lesion was performed.…”

Section: Case Report 2 Clinical Summarymentioning

confidence: 99%

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Pulmonary Crystal-Storing Histiocytosis

Urisman

Jones

2012

Pathology Case Reviews

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Pulmonary crystal-storing histiocytosis (CSH) is an uncommon condition that is most frequently associated with a clonal lymphoproliferative or plasma cell disorder (LPD/PCD). We present 2 cases of pulmonary CSH. The first case was diagnosed as an incidental finding during autopsy of a 77-year-old man with a 20-year history of idiopathic neutropenia, who died of complications of pancreatic adenocarcinoma. In this patient, CSH was associated with extranodal marginal zone lymphoma of bronchiole associated lymphoid tissue, and multifocal light chain deposition was found elsewhere in the lungs. The second case was diagnosed by core biopsy of an incidentally discovered lung mass in a 78-year-old woman with pneumonia. No associated LPD/PCD was identified in this patient. An additional 10 cases of localized pulmonary CSH were identified in the literature and are reviewed. Unlike generalized CSH, which virtually always occurs in the setting of multiple myeloma and is associated with poor long-term prognosis, localized disease confined to the lung seems to have good prognosis independent of whether there is an associated LPD/PCD and is cured by surgical resection in most of the cases. The histological diagnosis of CSH is made based on the presence of macrophages with characteristic eosinophilic intracytoplasmic needle-shaped or polygonal crystals composed of immunoglobulin proteins. Once the diagnosis of CSH is established, a diligent systemic workup to rule out generalized disease is indicated.A 77-year-old man with a 20-year history of idiopathic neutropenia was admitted to the hospital for evaluation of jaundice and bilirubin level of 13 mg/dL. Despite several hematologic evaluations, including 8 bone marrow biopsies, the etiology of the neutropenia was never determined. Endoscopic retrograde cholangiopancreatography showed a pancreatic duct stricture, and abdominal computed tomography (CT) revealed a pancreatic head mass suggestive of carcinoma. No evidence of local spread or metastases was identified, and the patient underwent a Whipple procedure. The pathological diagnosis confirmed the presence of pancreatic ductal adenocarcinoma. On postoperative day 10, the patient became hypoxemic and hypotensive. He developed methicillin-resistant Staphylococcus aureus septicemia, and abdominal CT was consistent with an intra-abdominal abscess. His condition worsened, and he died on postoperative day 16. Pathology GrossAutopsy confirmed the presence of intra-abdominal abscess and peritonitis. Examination of the lungs revealed numerous incidental firm yellow nodules up to 1 cm in diameter, with occasional cavitation, scattered throughout all lobes. In addition, a circumscribed spongy gray nodule of 2 cm in diameter was present in the left lower lobe. HistologySections of the firm yellow lesions (Fig. 1) showed nodular deposits of amorphous eosinophilic material suggestive of amyloid or nonamyloid light chain deposits. The nodules were rimmed by fibrosis with scattered foreign body-type multinucleated giant cells. There was an abr...

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Section: Review Of Published Casesmentioning

confidence: 80%

Section: Case Report 2 Clinical Summarymentioning

confidence: 99%

Pulmonary Crystal-Storing Histiocytosis

Urisman

Jones

2012

Pathology Case Reviews

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“…12 Human γD crystallin is a member of a highly homologous family of mammalian lens proteins called the γ crystallins. Together with the α and β crystallins, these proteins are essential for maintaining lens transparency.…”

Section: Protein Crystallization In Humanmentioning

confidence: 99%

“…In vivo-grown protein crystals have been observed from a varied group of organisms. 12,13,22,24 Positive natural selection REVIEW ARTICLE pressure acts on these proteins for in vivo crystallization due to their functional significance. In most cases, high protein concentration drives crystallization of these proteins inside the living organism.…”

Section: Introductionmentioning

confidence: 99%

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The New Era of Microcrystallography

et al. 2018

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Serial Femtosecond Crystallography: A Decade at the Forefront in Structural Biology

Fromme

Graves

Martín-García

2020

Encyclopedia of Life Sciences

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Exceptionally brilliant, femtosecond‐pulsed X‐ray sources, the X‐ray free‐electron lasers (XFELs), have brought a new way of conducting crystallography by probing nano/micrometre‐sized crystals in a serial fashion. Since the first XFEL, the Linac Coherent Light Source (LCLS), started operation in 2009, the serial femtosecond crystallography (SFX) technique has opened up new exciting opportunities for the determination of static structures as well as the structural dynamics of macromolecules. Here we gathers information from the greatest advances and exciting discoveries in the past 10 years in the emerging technology of SFX at XFELs as well as outlines the frontiers of this technology at upcoming compact pulsed X‐ray sources and its implementation at synchrotron radiation sources. Key Concepts Reviewed 10 years of successful science at X‐ray free‐electron lasers (XFELs). Highlighted the major key advances of the SFX technique in the areas of sample delivery and data analysis. Highlighted the breakthrough experiments to determine the structure of highly relevant therapeutic targets such as GPCRs. Highlighted the breakthrough experiments to study structural dynamics of macromolecules using the time‐resolved technique. Outlined the frontiers of serial crystallography technology at modern XFELs and compact instruments as well as its implementation at synchrotron radiation sources.

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Crystal-Storing Histiocytosis: Report of a Case, Review of the Literature (80 Cases) and a Proposed Classification

Cited by 127 publications

References 51 publications

Pulmonary Crystal-Storing Histiocytosis

Pulmonary Crystal-Storing Histiocytosis

The New Era of Microcrystallography

Serial Femtosecond Crystallography: A Decade at the Forefront in Structural Biology

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