The clinical spectrum of mycosis fungoides in Tanzania, East Africa

Grijsen, Marlous L.; Mtayangulwa, Regina; Naafs, Bernard; Jansen, Patty M.; Vermeer, Maarten H.; Mavura, Daudi; Masenga, Elisante J.; Willemze, Rein

doi:10.1111/bjd.14963

Cited by 6 publications

(3 citation statements)

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“…Several issues, including HIV coinfection, lack of access to specialty providers, and a relatively large dispersed rural population, may contribute to burden and late presentation of disease that can be seen in this setting. The literature available suggests that cutaneous lymphoma is likely under recognized and under diagnosed in sub‐Saharan Africa …”

Section: Introductionmentioning

confidence: 99%

A retrospective review of cutaneous lymphoma in Botswana

Rodriguez

Sowash

Mosojane³

et al. 2019

Int J Dermatology

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Background Primary cutaneous lymphoma (PCL) represents a heterogeneous collection of non-Hodgkin lymphomas originating in the skin. Our study describes the clinical and histological findings of cutaneous lymphoma within Botswana to expand the paucity of data on this rare disease in sub-Saharan Africa. Methods We conducted a retrospective review from the dermatology clinic at Princess Marina Hospital (Gaborone, Botswana) of patients evaluated by skin biopsy for cutaneous lymphoma between 2008 and 2017. Patients with initial diagnostic suspicion for cutaneous lymphoma had biopsies re-reviewed by experienced dermatopathologists and were given a final diagnosis of either (i) cutaneous lymphoma, (ii) atypical lymphocytic infiltrate (ALI), or (iii) a reactive cutaneous process. Results Thirty-eight cases were identified with a mean age of 50.0 years and a male: female (M:F) ratio of 13:6. Final diagnoses included: 27 cases of cutaneous lymphoma, eight cases of ALI, and three cases of reactive cutaneous processes. Subtypes of cutaneous lymphoma diagnosed included: mycosis fungoides (MF) (81.5%), plasmablastic lymphoma (7.4%), Epstein-Barr virus-positive T-cell lymphoma (3.7%), subcutaneous panniculitis-like T-cell lymphoma (3.7%), and peripheral T-cell lymphoma, not otherwise specified (3.7%). The most common immunohistochemical staining profile in MF cases was CD8 predominance over CD4.Conclusions Primary cutaneous lymphoma causes significant morbidity and mortality globally. Given the limited resources in sub-Saharan Africa, it is essential to educate providers on the manifestations and histology of PCL. This study is an important step towards understanding the demographics, clinical presentation, histologic features, and mortality of patients diagnosed with PCL in Botswana and similar low-resource settings.In order to further evaluate this disease in sub-Saharan Africa, our study reviewed the clinical and histological findings Two cases of plasmablastic lymphoma were included in this series. A 42-year-old HIV + male presented with painful penile and perineal nodules. In addition, a 32-year-old HIV + male presented with a large, exophytic, ulcerated, necrotic tumor on his right flank surrounded by an indurated plaque with multiple ª

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Section: Introductionmentioning

confidence: 99%

A retrospective review of cutaneous lymphoma in Botswana

Rodriguez

Sowash

Mosojane³

et al. 2019

Int J Dermatology

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“…This low reporting partly reflects the rarity of the disease, but is likely compounded by the very low awareness of HTLV-1 and ATL among local clinicians and by the absence of diagnostic tools (point 4). Such tools are needed to establish an accurate differential diagnosis between ATL and other hematologic malignancies, including cutaneous T-cell lymphomas, such as mycosis fungoides (184,207,208) or Seźary's syndrome (133,209), which are also present in African patients. An extensive search of medical publications over a period of almost 40 years identified only about 160 reported cases of ATL in patients of African origin (Table 5).…”

Section: Htlv-1-associated Diseases 41 What Do We Know About Adult T-...mentioning

confidence: 99%

“…Little is known about the situation in East Africa, but because some patients suffering from other types of cutaneous T-cell lymphomas have been reported in Rwanda (209) and Tanzania (207), we can assume that ATL is extremely rare in this region of low HTLV-1 endemicity (144).…”

Section: Htlv-1-associated Diseases 41 What Do We Know About Adult T-...mentioning

confidence: 99%

Geographic distribution, clinical epidemiology and genetic diversity of the human oncogenic retrovirus HTLV-1 in Africa, the world’s largest endemic area

et al. 2023

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The African continent is considered the largest high endemic area for the oncogenic retrovirus HTLV-1 with an estimated two to five million infected individuals. However, data on epidemiological aspects, in particular prevalence, risk factors and geographical distribution, are still very limited for many regions: on the one hand, few large-scale and representative studies have been performed and, on the other hand, many studies do not include confirmatory tests, resulting in indeterminate serological results, and a likely overestimation of HTLV-1 seroprevalence. For this review, we included the most robust studies published since 1984 on the prevalence of HTLV-1 and the two major diseases associated with this infection in people living in Africa and the Indian Ocean islands: adult T-cell leukemia (ATL) and tropical spastic paraparesis or HTLV-1-associated myelopathy (HAM/TSP). We also considered most of the book chapters and abstracts published at the 20 international conferences on HTLV and related viruses held since 1985, as well as the results of recent meta-analyses regarding the status of HTLV-1 in West and sub-Saharan Africa. Based on this bibliography, it appears that HTLV-1 distribution is very heterogeneous in Africa: The highest prevalences of HTLV-1 are reported in western, central and southern Africa, while eastern and northern Africa show lower prevalences. In highly endemic areas, the HTLV-1 prevalence in the adult population ranges from 0.3 to 3%, increases with age, and is highest among women. In rural areas of Gabon and the Democratic Republic of the Congo (DRC), HTLV-1 prevalence can reach up to 10-25% in elder women. HTLV-1-associated diseases in African patients have rarely been reported in situ on hospital wards, by local physicians. With the exception of the Republic of South Africa, DRC and Senegal, most reports on ATL and HAM/TSP in African patients have been published by European and American clinicians and involve immigrants or medical returnees to Europe (France and the UK) and the United States. There is clearly a huge underreporting of these diseases on the African continent. The genetic diversity of HTLV-1 is greatest in Africa, where six distinct genotypes (a, b, d, e, f, g) have been identified. The most frequent genotype in central Africa is genotype b. The other genotypes found in central Africa (d, e, f and g) are very rare. The vast majority of HTLV-1 strains from West and North Africa belong to genotype a, the so-called ‘Cosmopolitan’ genotype. These strains form five clades roughly reflecting the geographic origin of the infected individuals. We have recently shown that some of these clades are the result of recombination between a-WA and a-NA strains. Almost all sequences from southern Africa belong to Transcontinental a-genotype subgroup.

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Mycosis fungoides

2020

Skin Lymphoma

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The clinical spectrum of mycosis fungoides in Tanzania, East Africa

Cited by 6 publications

References 9 publications

A retrospective review of cutaneous lymphoma in Botswana

A retrospective review of cutaneous lymphoma in Botswana

Geographic distribution, clinical epidemiology and genetic diversity of the human oncogenic retrovirus HTLV-1 in Africa, the world’s largest endemic area

Mycosis fungoides

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