Eosinophilic Fasciitis with Hypereosinophilia as the Initial Clinical Manifestation of Peripheral T-Cell Lymphoma, Not Otherwise Specified

A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination....

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Published inInternal Medicine Vol. 61; no. 22; pp. 3425 - 3429
Main Authors Okuyama, Shuhei, Shishido, Tsutomu, Ueki, Toshimitsu, Sato, Keijiro, Satomi, Hidetoshi, Ishikawa, Ryuto, Sumi, Masahiko, Kobayashi, Hikaru
Format Journal Article
LanguageEnglish
Published Japan The Japanese Society of Internal Medicine 15.11.2022
Japan Science and Technology Agency
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Abstract A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.
AbstractList A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.
A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.
ArticleNumber 9300-21
Author Ueki, Toshimitsu
Satomi, Hidetoshi
Shishido, Tsutomu
Kobayashi, Hikaru
Ishikawa, Ryuto
Sato, Keijiro
Sumi, Masahiko
Okuyama, Shuhei
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  fullname: Shishido, Tsutomu
  organization: Department of Hematology, Nagano Red Cross Hospital, Japan
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  fullname: Ueki, Toshimitsu
  organization: Department of Hematology, Nagano Red Cross Hospital, Japan
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  fullname: Sato, Keijiro
  organization: Department of Hematology, Nagano Red Cross Hospital, Japan
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  fullname: Satomi, Hidetoshi
  organization: Department of Pathology, Nagano Red Cross Hospital, Japan
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Cites_doi 10.1080/03009740410005061
10.1056/NEJM199402243300804
10.1016/j.jaci.2012.02.019
10.1016/j.iac.2007.07.002
10.3346/jkms.2000.15.3.346
10.2214/ajr.184.3.01840975
10.3324/haematol.2014.118042
10.1200/JCO.1984.2.7.788
10.1097/DAD.0b013e3182892230
10.3109/10428199709059688
10.1182/blood-2016-01-643569
10.1016/j.jaci.2010.03.042
10.3389/fimmu.2020.01765
10.1016/j.berh.2012.08.001
10.1046/j.1365-2133.1998.02535.x
10.1097/MD.0000000000000088
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Issue 22
Keywords hematologic neoplasms
eosinophilia
eosinophilic fasciitis
hypereosinophilic syndrome
T-cell lymphoma
Language English
License The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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References 14. Carpentier C, Verbanck S, Schandené L, et al. Eosinophilia associated with CD3-CD4+ T cells: characterization and outcome of a single-center cohort of 26 patients. Front Immunol 11: 1765, 2020.
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11. Simon HU, Rothenberg ME, Bochner BS, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol 126: 45-49, 2010.
4. Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol 26: 449-458, 2012.
15. Lefèvre G, Copin MC, Staumont-Sallé D, et al. The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ aberrant T-cell phenotype. Medicine (Baltimore) 93: 255-266, 2014.
3. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 127: 2375-2390, 2016.
16. Lefèvre G, Copin MC, Roumier C, et al. CD3-CD4+ lymphoid variant of hypereosinophilic syndrome: nodal and extranodal histopathological and immunophenotypic features of a peripheral indolent clonal T-cell lymphoproliferative disorder. Haematologica 100: 1086-1095, 2015.
9. Masuoka H, Kikuchi K, Takahashi S, Kakinuma T, Hayashi N, Furue M. Eosinophilic fasciitis associated with low-grade T-cell lymphoma. Br J Dermatol 139: 928-930, 1998.
12. Cogan E, Schandené L, Crusiaux A, Cochaux P, Velu T, Goldman M. Brief report: clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome. N Engl J Med 330: 535-538, 1994.
7. Eklund KK, Anttila P, Leirisalo-Repo M. Eosinophilic fasciitis, myositis and arthritis as early manifestations of peripheral T-cell lymphoma. Scand J Rheumatol 32: 376-377, 2003.
1. Greer JP, York JC, Cousar JB, et al. Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases. J Clin Oncol 2: 788-798, 1984.
6. Castellanos-González M, Velasco Rodriguez D, Blanco Echevarría A, et al. Eosinophilic fasciitis as a manifestation of a cutaneous T-cell lymphoma not otherwise specified. Am J Dermatopathol 35: 666-670, 2013.
8. Kim H, Kim MO, Ahn MJ, et al. Eosinophilic fasciitis preceding relapse of peripheral T-cell lymphoma. J Korean Med Sci 15: 346-350, 2000.
2. Kitano K, Ichikawa N, Shimodaira S, Ito T, Ishida F, Kiyosawa K. Eosinophilia associated with clonal T-cell proliferation. Leuk Lymphoma 27: 335-342, 1997.
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12
13
14
15
16
1
2
3
4
5
6
7
8
9
10
References_xml – reference: 5. Moulton SJ, Kransdorf MJ, Ginsburg WW, Abril A, Persellin S. Eosinophilic fasciitis: spectrum of MRI findings. AJR Am J Roentgenol 184: 975-978, 2005.
– reference: 10. Valent P, Klion AD, Horny HP, et al. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol 130: 607-612.e609, 2012.
– reference: 3. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 127: 2375-2390, 2016.
– reference: 15. Lefèvre G, Copin MC, Staumont-Sallé D, et al. The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ aberrant T-cell phenotype. Medicine (Baltimore) 93: 255-266, 2014.
– reference: 7. Eklund KK, Anttila P, Leirisalo-Repo M. Eosinophilic fasciitis, myositis and arthritis as early manifestations of peripheral T-cell lymphoma. Scand J Rheumatol 32: 376-377, 2003.
– reference: 4. Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol 26: 449-458, 2012.
– reference: 8. Kim H, Kim MO, Ahn MJ, et al. Eosinophilic fasciitis preceding relapse of peripheral T-cell lymphoma. J Korean Med Sci 15: 346-350, 2000.
– reference: 11. Simon HU, Rothenberg ME, Bochner BS, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol 126: 45-49, 2010.
– reference: 12. Cogan E, Schandené L, Crusiaux A, Cochaux P, Velu T, Goldman M. Brief report: clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome. N Engl J Med 330: 535-538, 1994.
– reference: 13. Roufosse F, Cogan E, Goldman M. Lymphocytic variant hypereosinophilic syndromes. Immunol Allergy Clin North Am 27: 389-413, 2007.
– reference: 16. Lefèvre G, Copin MC, Roumier C, et al. CD3-CD4+ lymphoid variant of hypereosinophilic syndrome: nodal and extranodal histopathological and immunophenotypic features of a peripheral indolent clonal T-cell lymphoproliferative disorder. Haematologica 100: 1086-1095, 2015.
– reference: 2. Kitano K, Ichikawa N, Shimodaira S, Ito T, Ishida F, Kiyosawa K. Eosinophilia associated with clonal T-cell proliferation. Leuk Lymphoma 27: 335-342, 1997.
– reference: 14. Carpentier C, Verbanck S, Schandené L, et al. Eosinophilia associated with CD3-CD4+ T cells: characterization and outcome of a single-center cohort of 26 patients. Front Immunol 11: 1765, 2020.
– reference: 1. Greer JP, York JC, Cousar JB, et al. Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases. J Clin Oncol 2: 788-798, 1984.
– reference: 9. Masuoka H, Kikuchi K, Takahashi S, Kakinuma T, Hayashi N, Furue M. Eosinophilic fasciitis associated with low-grade T-cell lymphoma. Br J Dermatol 139: 928-930, 1998.
– reference: 6. Castellanos-González M, Velasco Rodriguez D, Blanco Echevarría A, et al. Eosinophilic fasciitis as a manifestation of a cutaneous T-cell lymphoma not otherwise specified. Am J Dermatopathol 35: 666-670, 2013.
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  doi: 10.2214/ajr.184.3.01840975
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  doi: 10.3109/10428199709059688
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  doi: 10.1182/blood-2016-01-643569
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  doi: 10.1016/j.jaci.2010.03.042
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  doi: 10.3389/fimmu.2020.01765
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Snippet A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for...
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SubjectTerms Biopsy
Bone marrow
Case Report
Diagnosis
Edema
Eosinophilia
Eosinophilia - complications
Eosinophilia - diagnosis
eosinophilic fasciitis
Fasciitis
Fasciitis - diagnosis
Fasciitis - drug therapy
Fasciitis - etiology
Hematologic Neoplasms
Histiocytosis
Humans
hypereosinophilic syndrome
Hypereosinophilic Syndrome - complications
Hypereosinophilic Syndrome - diagnosis
Internal medicine
Leukocytes (eosinophilic)
Liver diseases
Lymphocytes T
Lymphocytosis
Lymphoma, T-Cell, Peripheral - complications
Lymphoma, T-Cell, Peripheral - diagnosis
Male
Malignancy
Middle Aged
T-cell lymphoma
Title Eosinophilic Fasciitis with Hypereosinophilia as the Initial Clinical Manifestation of Peripheral T-Cell Lymphoma, Not Otherwise Specified
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