Novel Hepatosplenic T-Cell Lymphoma in Young Adult with Sickle Cell Disease

Case Report

Austin J Pathol Lab Med. 2019; 6(1): 1022.

Novel Hepatosplenic T-Cell Lymphoma in Young Adult with Sickle Cell Disease

Ghazal MA1* and ALHajjaj D2

1Department of Hematopathology, Dammam Regional Laboratory, Saudi Arabia

2Department of Medicine, Dammam Complex Hospital, MOH, KSA

*Corresponding author: Ghazal MA, Department of Hematopathology, Dammam Regional Laboratory, Saudi Arabia

Received: December 20, 2018; Accepted: January 30, 2019; Published: February 06, 2019


Hepatospenic T-cell Lymphoma (HSTL) is rare & an aggressive type of extranodal lymphoma, the disease represent 1-2% of all peripheral T-cell lymphoma [1]. Its incidence might be underestimated, because the disease may mimic other conditions & the diagnosis is sometimes difficult to be established .It has been seen in patients receiving long-term immunosuppressive therapy, following AML or EBV positive lymphoproliferative disorders or during pregnancy. Several cases were reported recently in patients with crohns disease treated with azathioprine & anti-tumor necrosis factor agent infliximab. We present here peculiar scenario of young male adult known case of sickle cell disease patient whose been diagnosed with gammadelta-T-cell lymphoma, after quite long history of unexplained organomegaly & fever.


HSTL is rare, and the world health organization prefers the term hepatosplenic T-cell lymphoma in the current WHO classification of tumors of haematopoietic & lymphoid tissues. The disease occurs mainly in young adults presenting with hepatomegaly, splenomegaly but without lymphadenopathy. Most patients have B-symptoms & cytopenias. In fact bone marrow involvement is nearly always present. Neoplastic cells infiltrate & expand the bone marrow sinuses, a feature that is highly characteristic & thus a useful diagnostic criterion, irrespective of their gammadelta or alphabeta phenotype, HSTL shows a clonal rearrangement of TCRG gene. Recently gene expression profiling studies have shown that the HSTL signature was distinct & characterized by over expression of gene encoding KIRs molecules. In conventional cytogenetic & FISH studies, 50-80% were characterized by the presence of an isochromsome 7q(i[7][q10]) this is occasionally the sole karyotypic abnormality suggesting that it plays a role in pathogenesis, in addition to trisomy 8 & loss of chromosome Y. The major differential diagnoses include aggressive NK-cell lymphoma/leukemia, T-Cell large granular lymphocytic leukemia & other gammadelta T-cell lymphomas.

Case Presentation

26 years old young male, known case of SCD on hydroxyurea, with history of multiple transfusion, was admitted on 19 September 2018 because of very high white blood cell count 91,000/ul with abnormal liver function test. Going back to his history, patient was admitted to another hospital 3 months back with abdominal pain, diagnosed as sequestration crisis at that time followed by splenctomy, postoperatively was found to have high grade fever reaching up to 40°C, CT abdomen was done & showed collection at site of operation, so explored & the patient was kept on antibiotics, but unfortunately he was continued to have fever, so bone marrow was done but was inadequate, however trephine biopsy showed grad III fibrosis & repetition was suggested. After splenctomy the patient decided to stop hydroxyurea, since then his white blood cells started to rise up. The patient gave history of significant weight loss (around 10KG). all septic work up done were negative.

On clinical examination, he was jaundiced, thin, tall, pale with fever of round 40°C. chest examination was clear while abdomen shows long longitudinal scar with huge hepatomegaly. There is daily increase in WBC counts, although he is covered with imipinem & hydroxyurea. Peripheral blood examination shows total white blood count of around 147,870/cumm. Hb-6.9 g/dl, Platelets-1340000/cumm next day total white cells rise to 161,320/cumm. While both hemoglobin & platelets counts dropped down to Hb-6.7 g/dl, & Platelets-930000/ cumm, PT &APTT are normal, however total bilurbin is elevated 22.11mg/dl(normal is less than 0.3mg/dl), mainly direct 16.4mg/dl(normal range is 0.3-1.0mg/dl), AST (GOT):381(normal is 10-40 units/L)., ALT(GPT):105(normal is 7-56 units/l).GGT:194U/L(15-85) LDH:2508U/L(normal range is (100-190), alkaline phosphastase is 427U/L(46-116).

Peripheral blood morphology examination shows Marked Leukocytosis, Leukoerythroblastic reaction, Nucleated RBC- 47/100 WBC. There is infiltration by around (75%) atypical lymphoid cells Majority are medium sized have moderate to abundant amount of cytoplasm some of them have round to oval nuclei with few granules resembling LGL cells, Others have mostly oval nuclei with open chromatin, one to two nucleoli (blast like morphology), (Figure 1).

Flow cytometry was conducted on peripheral blood and shows that majority of population are T-Lymphocytes (80%) Double Negative for CD4, CD8 and Positive for s-CD3, c-CD3, CD2, CD7, CD16, CD56, CD11b, CD11c & express gamma delta-T-cells receptor. They are Negative for CD34, TdT, HLA-DR, CD117, CD13, CD33, CD14, CD64, CD4, CD5, CD8, CD10, CD19, CD22, CD99, CD1a, CD25, CD20, CD79a, CD57, Alpha Beta and c-MPO, (Figure 2). Bone marrow studies were conducted, unfortunately was dry tap (both sides are tried), however bone Marrow Trephine Biopsy was adequate with increased cellularity of around 80-90% showing interstitial infiltration by variable sized lymphocytes, majority are small size with clumped chromatin along with moderate size lymphocytes with slightly open chromatin & prominent nucleoli. Myeloid series are suppressed while erythroid & megakaryocytes are present, (Figure 3).