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Multifaceted adult T-cell leukemia/lymphoma in India: a case series.

Khader A, Shaan M, Balakrishnan S, Ambooken B, Muhammed K, Rajan U - Indian J Dermatol (2015 Jan-Feb)

Bottom Line: Presence of epidermotropism did not correlate with the severity of disease.ATL manifest in myriad presentations and skin lesions are often the earliest manifestation.Cutaneous manifestations of ATL vary from subtle hypopigmented macules to florid nodular lesions, and HTLV-1 screening need to be carried out in all doubtful cases.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Government Medical College, Calicut, Kerala, India.

ABSTRACT

Background: Adult T-cell leukemia/lymphoma (ATL) is caused by human T-cell lymphotropic virus type-1 (HTLV-1). India is considered as a nonendemic region for HTLV-1. Recent upsurge of cases have been noted in southern parts of India.

Aims and objectives: The objective was to describe skin manifestations in various types of ATL.

Materials and methods: Clinical examination, blood investigations, skin biopsies, lymph node biopsies, and immunohistochemistry were performed in five patients. Flow cytometry was performed in two cases.

Results: Serological testing was positive for HTLV-1 in all patients. All patients presented with skin lesions. Rare presentations of molluscum contagiosum like papules, purpuric macules and plaques, hypopigmented macules and verrucous papules were seen. Dermatophytic infections occurred in two patients. Mucosal lesion was seen in one patient. Histological features include dermal lymphoid infiltrate with or without epidermotropism. Presence of epidermotropism did not correlate with the severity of disease. All patients except one succumbed to illness within few months to 1 year period.

Conclusions: ATL manifest in myriad presentations and skin lesions are often the earliest manifestation. Cutaneous manifestations of ATL vary from subtle hypopigmented macules to florid nodular lesions, and HTLV-1 screening need to be carried out in all doubtful cases.

No MeSH data available.


Related in: MedlinePlus

Peripheral smear of patient 2 showing atypical lymphoid cells with indented nuclei (hematoxylin and eosin stain (H and E), magnification 100×)
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Figure 3: Peripheral smear of patient 2 showing atypical lymphoid cells with indented nuclei (hematoxylin and eosin stain (H and E), magnification 100×)

Mentions: A 32-year-old male with history of hypopigmented macules over chest and trunk of 1 year duration, presented with pruritic purpuric macules and plaques over face, trunk, and extremities and purpuric annular plaque over medial aspect of thighs of 20 days duration [Figure 1]. Wet purpura was present over hard palate and buccal mucosa [Figure 2]. Patient had submandibular, cervical, epitrochlear, and inguinal lymph node enlargement. Scraping for fungus from the annular purpuric plaque revealed plenty of hyphae. His total leukocyte count was elevated and peripheral smear revealed large number of atypical lymphocytes with cleaved nucleus [Figure 3] which were CD3+, CD5+, CD 25+, and CD7- by flow cytometry. Serum calcium, serum LDH, blood urea, and serum ALP were elevated. Platelet count was 2.04 lakhs/mm3 and serum albumin 2.7 g. Dense infiltrate of medium sized atypical lymphocytes occupied the dermis with epidermotropism [Figure 4] in skin biopsy and similar infiltrate in lymph node biopsy which showed CD3+, CD4+, CD8-, and CD20- on immunohistochemistry. Patchy infiltrate of atypical lymphoid cells with irregular nuclei were found in the bone marrow trephine biopsy. Ultrasonography of abdomen showed bilateral bulky kidneys with raised echoes and loss of sinus fat and bilateral pleural effusion. Skull X-ray showed lytic lesions. HTLV-1 antibody was positive by ELISA and screening for HIV negative.


Multifaceted adult T-cell leukemia/lymphoma in India: a case series.

Khader A, Shaan M, Balakrishnan S, Ambooken B, Muhammed K, Rajan U - Indian J Dermatol (2015 Jan-Feb)

Peripheral smear of patient 2 showing atypical lymphoid cells with indented nuclei (hematoxylin and eosin stain (H and E), magnification 100×)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4318027&req=5

Figure 3: Peripheral smear of patient 2 showing atypical lymphoid cells with indented nuclei (hematoxylin and eosin stain (H and E), magnification 100×)
Mentions: A 32-year-old male with history of hypopigmented macules over chest and trunk of 1 year duration, presented with pruritic purpuric macules and plaques over face, trunk, and extremities and purpuric annular plaque over medial aspect of thighs of 20 days duration [Figure 1]. Wet purpura was present over hard palate and buccal mucosa [Figure 2]. Patient had submandibular, cervical, epitrochlear, and inguinal lymph node enlargement. Scraping for fungus from the annular purpuric plaque revealed plenty of hyphae. His total leukocyte count was elevated and peripheral smear revealed large number of atypical lymphocytes with cleaved nucleus [Figure 3] which were CD3+, CD5+, CD 25+, and CD7- by flow cytometry. Serum calcium, serum LDH, blood urea, and serum ALP were elevated. Platelet count was 2.04 lakhs/mm3 and serum albumin 2.7 g. Dense infiltrate of medium sized atypical lymphocytes occupied the dermis with epidermotropism [Figure 4] in skin biopsy and similar infiltrate in lymph node biopsy which showed CD3+, CD4+, CD8-, and CD20- on immunohistochemistry. Patchy infiltrate of atypical lymphoid cells with irregular nuclei were found in the bone marrow trephine biopsy. Ultrasonography of abdomen showed bilateral bulky kidneys with raised echoes and loss of sinus fat and bilateral pleural effusion. Skull X-ray showed lytic lesions. HTLV-1 antibody was positive by ELISA and screening for HIV negative.

Bottom Line: Presence of epidermotropism did not correlate with the severity of disease.ATL manifest in myriad presentations and skin lesions are often the earliest manifestation.Cutaneous manifestations of ATL vary from subtle hypopigmented macules to florid nodular lesions, and HTLV-1 screening need to be carried out in all doubtful cases.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Government Medical College, Calicut, Kerala, India.

ABSTRACT

Background: Adult T-cell leukemia/lymphoma (ATL) is caused by human T-cell lymphotropic virus type-1 (HTLV-1). India is considered as a nonendemic region for HTLV-1. Recent upsurge of cases have been noted in southern parts of India.

Aims and objectives: The objective was to describe skin manifestations in various types of ATL.

Materials and methods: Clinical examination, blood investigations, skin biopsies, lymph node biopsies, and immunohistochemistry were performed in five patients. Flow cytometry was performed in two cases.

Results: Serological testing was positive for HTLV-1 in all patients. All patients presented with skin lesions. Rare presentations of molluscum contagiosum like papules, purpuric macules and plaques, hypopigmented macules and verrucous papules were seen. Dermatophytic infections occurred in two patients. Mucosal lesion was seen in one patient. Histological features include dermal lymphoid infiltrate with or without epidermotropism. Presence of epidermotropism did not correlate with the severity of disease. All patients except one succumbed to illness within few months to 1 year period.

Conclusions: ATL manifest in myriad presentations and skin lesions are often the earliest manifestation. Cutaneous manifestations of ATL vary from subtle hypopigmented macules to florid nodular lesions, and HTLV-1 screening need to be carried out in all doubtful cases.

No MeSH data available.


Related in: MedlinePlus