Acute HTLV-1 leukemia / lymphoma in a 33 year old grenadian migrant : A case report

On examination she was found to be icteric and with pale mucous membrane. Her pulse was 80/min, blood pressure 90/60 mm Hg, respiratory rate 15/min and temperature 36.5oC. She was not in discomfort. She had generalized nodules distributed on the upper and lower limbs, chest and face (Figs. 1 and 2). They were non-erythematous, and non-tender. No cervical, axillary or inguinal lymph nodes were palpable.

The neoplastic process presents in the early stages with a skin lesion as the primary site [5,6] and progresses to the advance stages with multiple organs involvement of the reticuloendothelial system such as the liver, spleen, and, bone marrow.There are indeed other presentations as in the case in discussion that presented as an acute leukemia with diffuse skin involvement.

CASE REPORT
A 33-year-old Grenadian national was on a six months visit to Trinidad when she developed a generalized pruritic skin rash and abdominal pain following consumption of shrimps.She visited the General Hospital for the above complain three days following the above symptoms.
On examination she was found to be icteric and with pale mucous membrane.Her pulse was 80/min, blood pressure 90/60 mm Hg, respiratory rate 15/min and temperature 36.5 o C.She was not in discomfort.She had generalized nodules distributed on the upper and lower limbs, chest and face (Figs. 1 and 2).They were non-erythematous, and non-tender.No cervical, axillary or inguinal lymph nodes were palpable.She was mildly tender in the left hypochondrium, with a markedly palpable spleen.
Her genitourinary and central nervous systems examination were essentially normal.She was started on analgesic and antipruritic oral medications and was admitted to the medical ward for further investigation.
The lymphocytes expressed T-cell markers with CD3, CD5, and CD4 positivity and were negative for CD7 and CD34.
Her HTLV-1 was positive.She was started on hydroxyurea and referred to the oncologist for further management.
The patient died one month after diagnosis while undergoing chemotherapy.

DISCUSSION
Human T-cell Lymphotrophic virus-1 is endemic in the Caribbean, Japan [7,8] and is seen now in most countries amongst immigrants from endemic countries [9,10].
The virus causes HAM/TSP and is the causative agent of HTLV-1 leukemia/lymphoma [1].
The acute, chronic, smoldering and lymphoma are the four different clinical manifestations of the neoplasm that have been identified [2].We describe here a Grenadian national who presented in Trinidad W.I with splenomegaly and an acute HTLV-1 leukemia, having a white blood cell count of 131 x 10^3/UL with 83.6% atypical lymphocytes within the peripheral blood and a diffuse nodular skin lesion which histologically contained malignant HTLV-1 infiltrate.Hairy cell leukemia and chronic myeloid leukemia both have splenomegaly of the magnitude seen in this patient.However these two leukemia were eliminated from the diagnosis on account of the HTLV-1 positivity, the immuonhistology of the malignant cells, the absence of hairy cells and myeloblast in the peripheral blood and bone marrow aspirate.The skin nodules were composed of malignant infiltrate of HTLV-1 cells, which had no correlation with the consumption of shrimps.In addition to the lymphocytosis, she also presented with hypercalcaemia and a markedly elevated LDH, which are poor prognostic markers.This patient only survived one month after diagnosis.
HTLV-1 skin manifestation are varied and includes infective dermatitis, maculo-papular hyper pigmented or hypo pigmented rashes, nodules or ill-defined rashes, which mimics Mycosis fungoides, Psoriasis, Eczema and a list of common dermatoses, [11] and may be an early presentation of this neoplastic disease entity [5,6].
Clinicians should have a high index of suspicion of HTLV-1 related disease in patients from endemic regions who presents with a skin rash.

CONCLUSION
HTLV-1 is endemic in the Caribbean and Japan.Immigrants presenting with a skin rash from endemic regions should be investigated for HTLV-1 Leukemia/ Lymphoma and it's other related diseases [12].

Figure 1 :
Figure 1: Skin nodules in the lower extremities.

Figure 3 :
Figure 3: The sections show skin with hyperkeratosis.Within the dermis is a nodular infi ltrate of atypical lymphocytes which is separated from the epidermis by a grenz.zone.

Figure 4 :
Figure 4: At higher magnifi cation the atypical infi ltrate was that of a T-cell lymphoma which is composed of small and medium size lymphocytes with pleomorphic nuclei and sparse eosinophilic nucleoli.Mitoses were infrequent.Admixed with these lymphoma cells were histiocytic cells.