A Rare Case of Leukemia Cutis in T-cell Acute Lymphoblastic Leukemia (T-ALL) in an Elderly Patient

Primary cutaneous involvement in T-cell acute lymphoblastic leukemia (ALL) is rare in elderly patients. Here we present a case of 65 years old male admitted to a metropolitan tertiary care hospital because of maculo-papular lesions on chest and back for last 3 months as chief complaint along with on and off history of fever, fatigability and anorexia. On general examination, there was no lymphadenopathy. Per-abdomen examination revealed hepatosplenomegaly. CT scan of chest and abdomen showed para-tracheal, pre-tracheal, carinal, hilar and abdominal lymphadenopathy. Peripheral blood smear and bone marrow aspiration showed more than 80% of lymphoblasts. Flow cytometry of the bone marrow aspirate showed CD7, CD5, CD45 and TdT positivity indicating T–cell origin. Skin biopsy from maculo-papular lesion revealed secondary leukemic infiltration of the skin. Corroborating all the above findings, a final diagnosis of T-cell ALL with leukemia cutis was made. Thereafter patient was given chemotherapy and after one month, the skin lesions started resolving and total leukocyte count (TLC) decreased. One week later, skin lesions relapsed with fall in hemoglobin and rise in TLC. Repeat bone marrow aspirate showed 90% blasts cells. Then, patient was put on palliative therapy but his condition progressively deteriorated and ultimately died within four months of diagnosis. Case Report *Corresponding author: Dr.AnshuGupta, Associate Professor, Emergency Laboratory, Institute of Human Behaviour and Allied Sciences, Dilshad Garden, Delhi-110095, India. Phone: +91 9868396812 E-mail: [email protected] C-176 TALL with Leukemia Cutis in an Elderly Patient Annals of Pathology and Laboratory Medicine, Vol. 03, No. 04, October December 2016 Introduction Acute lymphoblastic leukemia (ALL) is a malignancy of bone marrow where normal hematopoietic cells are replaced by proliferating lymphoid precursor cells. It is the most common type of leukemia seen in paediatric age group constituting about 75% of all cases of leukemia [1] .Incidence of ALL in elderly patients (> 65 years) is 1-1.6 per 100000 patients[2].Among all newly diagnosed cases of ALL, approximately 11.2% were from age group of >65 years. WHO classifies ALL as Pre (precursor) B-lymphoblastic ALL (most common ALL in adults), Mature B cell ALL (Burkitt ALL corresponds to ALL-L3) and Pre (precursor) T-cell ALL[3]. Leukemia Cutis (LC) is extra-medullary manifestation of leukemia caused by infiltration of skin by neoplastic leukemic cells and thus resulting in clinically identifiable skin lesions[4]. Incidence of LC is more common in AML constituting 10-15% of cases as compared to chronic myeloproliferative disorders[5]. However LC is rare in cases of precursor Bor T-cell lymphoblastic leukemia/ lymphomas (1%)[6]. Here, we are reporting a case of T-cell acute lymphoblastic leukemia with cutaneous infiltration in an elderly, posing as a diagnostic challenge because of its unusual presentation and rare immunophenotype. Case Report A previously healthy 65 years old male was admitted to a metropolitan tertiary care hospital with chief complaints of fever, fatigue, anorexia, and pruritic maculo-papular lesions over chest and back for past 3 months. On clinical examination, the patient was averagely built and poorly nourished. Skin lesions weremaculo-papular, erythematous, non-tender without ulceration or induration and blanching with pressure (Fig.1).There was no palpable lymphadenopathy in cervical, axillary or inguinal region. Per-abdomen examination showed enlarged liver, 3cms below costal margin and moderate splenomegaly. Biochemistry investigations showed ALT: 45 IU/L, AST: 48 IU/L, LDH: 392 IU/L, with normal kidney function tests. Ultrasonography revealed hepatosplenomegaly with liver measuring 18 cm and spleen measuring 19 cm. Chest roentgenogram and bone scan were normal. Routine hematological investigations revealed haemoglobin: 11.5g/ dl and total leucocyte count (TLC): 55,500/cmm. Differential Leukocyte count was blast cells 80%, neutrophils 10%, lymphocytes 6%, monocytes 2%, eosinophils 2%, basophils 0%, and platelet count: 1,20,000/cmm. Leishman stained peripheral smear showed 80% of blasts which were small to intermediate in size and round with scant light-blue cytoplasm, moderately condensed to dispersed chromatin and inconspicuous nucleoli (Fig.2& 3). CT scan of the chest showed right para-tracheal, pre-tracheal, carinal and bilateral hilar lymphadenopathy.CT scan of the abdomen revealed hepatosplenomegaly and discrete pre and para-aortic and aorto-caval lymphadenopathy. Geimsa stained bone marrow aspirate smears showed more than 80% of blast cells that had characteristics of lymphoblasts. Special cytochemical stains i.e. Myeloperoxidase, Periodic acid schiff (PAS) and Sudan black stains were negative on bone marrow aspirate.Skin biopsy performed from one of the maculo-papular lesions showed thinned out epidermis. Thedermisshowed infiltration by atypical immature and blast like cells arranged in nodules, around blood vessels and adenexal structures (Fig.4& 5).These tumour cells were positive for CD45, suggestive of secondary leukemic infiltration of skin. On flowcytometry, CD7, CD5, CD45 and TdT were positive on bone marrow aspirate while CD10, HLADR, CD34 and B cell markers (CD19 and CD20) were negative. With these findings, a final diagnosis of immunologically T-cell type ALL with leukemia cutis was made. Patient was put on chemotherapy. Initially, skin lesions started resolving along with decrease in total leukocyte count (TLC) after one month of treatment but one week later, skin lesions relapsed started appearing on post-auricular region and then spreading over to rest of the body. His hemoglobin dropped to 6gm/dl, TLC increased and bone marrow examination showed 90% of blast cells. Patient was put on palliative therapy only because of poor response. His condition started deteriorating progressively, ultimately leading to death within four months of diagnosis. Fig. 1: Photograph showing maculo-papular lesions on chest of the patient