Contemporary classification of histiocytic disorders

Favara, Blaise E.; Feller, Alfred C.; Pauli, Macro; Jaffe, Elaine S.; Weiss, Lawrence M.; Aricò, Maurizio; Bucsky, Peter; Egeler, R. Maarten; Elinder, Göran; Gadner, Helmut; Gresik, Mary V.; Henter, Jan‐Inge; Imashuku, Shinsaku; Janka‐Schaub, Gritta; Jaffe, Randal C.; Ladisch, Stephan; Nézelof, C; Pritchard, Jon

doi:10.1002/(sici)1096-911x(199709)29:3<157::aid-mpo1>3.0.co;2-c

Cited by 741 publications

(214 citation statements)

References 49 publications

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“…In these diseases, FDG uptake is believed to be related to the high density of activated macrophages present in inflammatory lesions. While there is ongoing debate regarding the exact origin of the histiocytes in ECD (25,26), these cells are of the monocyte/ macrophage/dendritic cell lineages (27,28). This may account for the observed uptake in other regions.…”

Section: Discussionmentioning

confidence: 99%

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Arnaud¹,

Malek²,

Archambaud³

et al. 2009

Arthritis & Rheumatism

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Objective. Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. The aim of this study was to assess the value of whole-body scanning with 18 F-fluorodeoxyglucose-positron emission tomography (FDG-PET) in a large cohort of ECD patients from a single center.Methods. We retrospectively reviewed all PET scans performed on 31 patients with ECD who were referred to our department between 2005 and 2008. PET images were reviewed by 2 independent nuclear medicine specialist physicians and were compared with other imaging modalities performed within 15 days of each PET scan.Results. Thirty-one patients (10 women and 21 men; median age 59.5 years) underwent a total of 65 PET scans. Twenty-three patients (74%) were untreated at the time of the initial PET scan, whereas 30 of the 34 followup PET scans (88%) were performed in patients who were undergoing immunomodulatory therapy. Comparison of the initial and followup PET scans with other imaging modalities revealed that the sensitivity of PET scanning varied greatly among the different organs studied (range 4.3-100%), while the specificity remained high (range 69.2-100%). Followup PET scans were particularly helpful in assessing central nervous system (CNS) involvement, since the PET scan was able to detect an early therapeutic response of CNS lesions, even before magnetic resonance imaging showed a decrease in their size. PET scanning was also very helpful in evaluating the cardiovascular system, which is a major prognostic factor in ECD, by assessing the heart and the entire vascular tree during a single session.Conclusion. The results of our large, singlecenter, retrospective study suggest that the findings of a FDG-PET scan may be interesting in the initial assessment of patients with ECD, but its greater contribution is in followup of these patients.

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Section: Discussionmentioning

confidence: 99%

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Arnaud¹,

Malek²,

Archambaud³

et al. 2009

Arthritis & Rheumatism

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“…Thus, this group of human diseases demonstrates overlapping clinical and pathologic features with the findings for the dogs of the current study. 1,2 Finally, hypersplenism as described in humans is defined by four criteria: 1) cytopenias of one or more peripheral blood cell lines, 2) bone marrow hyperplasia, 3) splenomegaly, and 4) correction of cytopenia following splenectomy. At the present time, however, there is no agreement regarding the specific disorders included in the category, and some disorders for which there is agreement do not fulfill all four criteria.…”

Section: Discussionmentioning

confidence: 99%

Splenic Myeloid Metaplasia, Histiocytosis, and Hypersplenism in the Dog (65 Cases)

Spangler

Kass

1999

Vet Pathol

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Abstract. Splenectomy specimens from 65 dogs with severe, diffuse, sustained, and progressive splenomegaly were examined. The clinical signs, hematology, and serum chemistry values in for the dogs were not useful diagnostic features. Microscopic changes in the spleens were distinctive and consisted of 1) myeloid metaplasia, 2) histiocytosis, 3) erythrophagocytosis, and 4) thrombosis with segmental infarction. Ultrastructural features suggested proliferative changes in the splenic reticular cells and macrophages (reticular meshwork) that described a continuum from reactive changes associated with immunologic damage of erythrocytes to neoplastic proliferation of histiocytic components. Thirty percent of the dogs survived 12 months. Approximately one half (53%) of the dogs with complete postmortem evaluations showed multiorgan involvement with a tissue distribution and cell morphology consistent with histiocytic neoplasia. For the remaining dogs (47%), only splenic pathology was consistently present, and a specific cause of death was often not evident. Distinctive histologic changes in the splenic tissues-including mitotic activity, erythrophagocytosis, giant cell formation, thrombosis/ infarction, and the proportion and distribution of histiocytic and hematopoietic cells-were statistically evaluated for prognostic relevance. The presence of giant cells was the only reliable prognostic feature, and that was indicative of a fatal outcome. These descriptive changes of myeloid metaplasia in the canine spleen are compared with the human clinical and pathologic syndromes of 1) agnogenic myeloid metaplasia, 2) hemophagocytic syndromes, and 3) hypersplenism. These diseases in humans produce histopathologic changes in the spleen that are similar to those observed in the canine splenic tissue we examined in this study.

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“…Percutaneous needle biopsy of the S1 lesion under CT guidance stained positive for CD-1a and S-100. A diagnosis of LCH was made according to Histiocyte Society guidelines [9]. The patient had chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) and local radiotherapy.…”

Section: Casementioning

confidence: 99%

Langerhans cell histiocytosis with multiple spinal involvement

et al. 2010

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To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four soft tissue involvement and three had posterior arch extension. Compiling data from the eight largest case series of the spinal LCH reveals that 27.2% multiple vertebrae lesions. In these 55 cases, there were 26 female and 29 male with the mean age of 7.4 years (range 0.2-37). A total of 182 vertebrae were involved including 28.0% in the cervical spine, 47.8% in thoracic and 24.2% in the lumbar spine. Extraspinal LCH lesion was documented in 54.2% cases, visceral involvement in 31.1% and vertebra plana in 50% cases. Paravertebral and epidural extension were not documented in most cases. Pathological diagnosis was achieved in 47 cases including 8 open spine biopsy. The treatment strategy varied depending on different hospitals. One patient died, two had recurrence and the others had no evidence of the disease with an average of 7.2 years (range 1-21) of follow-up. Asymptomatic spinal lesions could be simply observed with or without bracing and chemotherapy is justified for multiple lesions. Surgical decompression should be reserved for the uncommon cases in which neurologic compromise does not respond to radiotherapy or progresses too rapidly for radiotherapy.

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Contemporary classification of histiocytic disorders

Cited by 741 publications

References 49 publications

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Splenic Myeloid Metaplasia, Histiocytosis, and Hypersplenism in the Dog (65 Cases)

Langerhans cell histiocytosis with multiple spinal involvement

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Contemporary classification of histiocytic disorders

Cited by 741 publications

References 49 publications

18F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

18F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Splenic Myeloid Metaplasia, Histiocytosis, and Hypersplenism in the Dog (65 Cases)

Langerhans cell histiocytosis with multiple spinal involvement

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Resources

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¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease