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Angioimmunoblastic T-cell lymphoma: a diagnostic challenge.

Ocampo-Garza J, Herz-Ruelas ME, González-Lopez EE, Mendoza-Oviedo EE, Garza-Chapa JI, Ocampo-Garza SS, Vázquez-Herrera NE, Miranda-Maldonado I, Ocampo-Candiani J - Case Rep Dermatol (2014)

Bottom Line: Its pathogenesis is still unclear, but in some cases it has been associated with infection, allergic reaction or drug exposure.We present the illustrative case of a 55-year-old woman with an AITL who presented prominent skin findings, arthritis, lymphadenopathy and hypereosinophilia.Chemotherapy with CHOP-21 and thalidomide was given, accomplishing complete remission after six cycles.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, University Hospital 'Dr. José Eleuterio González', Universidad Autónoma de Nuevo León, Monterrey, Mexico.

ABSTRACT
Angioimmunoblastic T-cell lymphoma (AITL) accounts for 15-20% of all peripheral T-cell lymphomas. It is a rare subtype of CD4 T-cell peripheral lymphoma that affects aged individuals, causing B symptoms, generalized lymphadenopathy and hepatosplenomegaly. Its pathogenesis is still unclear, but in some cases it has been associated with infection, allergic reaction or drug exposure. The majority of patients are diagnosed in an advanced stage and anthracycline based regimen is considered the first-line therapy. Skin involvement is not well characterized, occurring in up to 50% of patients and presenting as nonspecific rash, macules, papules, petechiae, purpura, nodules and urticaria. We present the illustrative case of a 55-year-old woman with an AITL who presented prominent skin findings, arthritis, lymphadenopathy and hypereosinophilia. Skin biopsy reported a T-cell lymphoma and the diagnosis of AITL was confirmed by an axillary lymph node biopsy, which was also positive for Epstein-Barr virus. Chemotherapy with CHOP-21 and thalidomide was given, accomplishing complete remission after six cycles.

No MeSH data available.


Related in: MedlinePlus

Multiple 2–4-mm erythematous, blanchable macules and papules on the patient's trunk and extremities.
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Figure 1: Multiple 2–4-mm erythematous, blanchable macules and papules on the patient's trunk and extremities.

Mentions: Physical examination revealed multiple 2–4-mm erythematous, blanchable macules and papules on her trunk and extremities (fig. 1). She also had bilateral arthritis of the interphalangeal joints of the hands, wrists, elbows and knees as well as multiple enlarged axillary and inguinal lymph nodes. She presented with hypereosinophilia with an absolute count of 47,600/μl. Bone marrow aspirate showed hypercellularity, with a myeloid:erythroid ratio of 5:1; 65% of the cells were eosinophilic precursors with normal maturation. Eight-color flow cytometry revealed a population of immature T cells comprising 10.4% of total white blood cells with the following markers: CD3+, CD4–, CD5+, CD7+ dim, CD8–, CD34–, CD45+ and TdT–. A lesional skin biopsy reported T-cell lymphoma (diffusely positive CD3, CD4 and CD5 cells and focally positive CD8 and TIA-1 cells) (fig. 2). An axillary lymph node biopsy was consistent with AITL (CD3, CD4, CD5, CD7 and Epstein-Barr virus positive and CD8 and CD10 negative, with a CD21+ dendritic cell expansion) (fig. 3). A CT scan of the chest and abdomen was negative for mediastinal or abdominal adenopathy.


Angioimmunoblastic T-cell lymphoma: a diagnostic challenge.

Ocampo-Garza J, Herz-Ruelas ME, González-Lopez EE, Mendoza-Oviedo EE, Garza-Chapa JI, Ocampo-Garza SS, Vázquez-Herrera NE, Miranda-Maldonado I, Ocampo-Candiani J - Case Rep Dermatol (2014)

Multiple 2–4-mm erythematous, blanchable macules and papules on the patient's trunk and extremities.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4307011&req=5

Figure 1: Multiple 2–4-mm erythematous, blanchable macules and papules on the patient's trunk and extremities.
Mentions: Physical examination revealed multiple 2–4-mm erythematous, blanchable macules and papules on her trunk and extremities (fig. 1). She also had bilateral arthritis of the interphalangeal joints of the hands, wrists, elbows and knees as well as multiple enlarged axillary and inguinal lymph nodes. She presented with hypereosinophilia with an absolute count of 47,600/μl. Bone marrow aspirate showed hypercellularity, with a myeloid:erythroid ratio of 5:1; 65% of the cells were eosinophilic precursors with normal maturation. Eight-color flow cytometry revealed a population of immature T cells comprising 10.4% of total white blood cells with the following markers: CD3+, CD4–, CD5+, CD7+ dim, CD8–, CD34–, CD45+ and TdT–. A lesional skin biopsy reported T-cell lymphoma (diffusely positive CD3, CD4 and CD5 cells and focally positive CD8 and TIA-1 cells) (fig. 2). An axillary lymph node biopsy was consistent with AITL (CD3, CD4, CD5, CD7 and Epstein-Barr virus positive and CD8 and CD10 negative, with a CD21+ dendritic cell expansion) (fig. 3). A CT scan of the chest and abdomen was negative for mediastinal or abdominal adenopathy.

Bottom Line: Its pathogenesis is still unclear, but in some cases it has been associated with infection, allergic reaction or drug exposure.We present the illustrative case of a 55-year-old woman with an AITL who presented prominent skin findings, arthritis, lymphadenopathy and hypereosinophilia.Chemotherapy with CHOP-21 and thalidomide was given, accomplishing complete remission after six cycles.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, University Hospital 'Dr. José Eleuterio González', Universidad Autónoma de Nuevo León, Monterrey, Mexico.

ABSTRACT
Angioimmunoblastic T-cell lymphoma (AITL) accounts for 15-20% of all peripheral T-cell lymphomas. It is a rare subtype of CD4 T-cell peripheral lymphoma that affects aged individuals, causing B symptoms, generalized lymphadenopathy and hepatosplenomegaly. Its pathogenesis is still unclear, but in some cases it has been associated with infection, allergic reaction or drug exposure. The majority of patients are diagnosed in an advanced stage and anthracycline based regimen is considered the first-line therapy. Skin involvement is not well characterized, occurring in up to 50% of patients and presenting as nonspecific rash, macules, papules, petechiae, purpura, nodules and urticaria. We present the illustrative case of a 55-year-old woman with an AITL who presented prominent skin findings, arthritis, lymphadenopathy and hypereosinophilia. Skin biopsy reported a T-cell lymphoma and the diagnosis of AITL was confirmed by an axillary lymph node biopsy, which was also positive for Epstein-Barr virus. Chemotherapy with CHOP-21 and thalidomide was given, accomplishing complete remission after six cycles.

No MeSH data available.


Related in: MedlinePlus