Case Report - (2022) Volume 8, Issue 2
Chronic Lymphocytic Leukemia (CLL) is characterized by presence of abnormal monoclonal lymphocytes, circulating in blood that may infiltrate all organs, particularly those of lymphoreticular system. This disorder has a heterogeneous clinical course. Enlarged lymph nodes are the classical revealing manifestation. Gastrointestinal (GI) involvement in CLL, especially obstructive jaundice, is uncommonly reported, ranging from 5.7% to 13%. We report an isolated and unusual gastrointestinal involvement of CLL in an older patient. A 72-year-old man with Hepatitis C virus (HCV) infection presented an obstructive jaundice, revealing a Chronic Lymphocytic Leukemia (CLL) infiltration of hepatic hilar lymph nodes without another lymphadenopathy. CT-guided biopsy of lymph node mass confirmed neoplastic B-cell nature. Rituximab and Reduced Dose CHOP (R-miniCHOP) protocol, after plastic biliary stent placement, was established concomitantly with Direct anti-viral drugs (DAAs). The patient was achieving sustained virological response. After 6 cycles of chemotherapy, restaging studies revealed disappearance of hepatic hilar lymphadenopathy in CT-scan, performed 12 months later. The present case is an example of a rare presentation of CLL. It is extremely important to keep in mind the possibility of leukemic infiltrate, in case of unexplained intra-abdominal mass, especially in patients with HCV infection, and to highlight the probability to have a complete response to chemotherapy associated with DAAs.
Chronic lymphocytic leukemia • obstructive jaundice • hepatitis C virus infection
Chronic lymphocytic leukemia (CLL), the most common form of leukemia in Western world, is a CD 5 B-cell neoplastic disorder characterized by presence of small, mature-appearing lymphocytes, circulating in blood that may infiltrate all organs, particularly those of lymphoreticular system [1,2]. CLL occurs in elderly patients and has a heterogeneous clinical course, for which treatment decision still counts on conventional parameters [3,4] Clinically, it is characterized by enlarged lymph nodes, most often in neck, armpit, or groin areas [5]. Gastrointestinal (GI) involvement in CLL, especially obstructive jaundice, is uncommonly reported, ranging from 5.7% to 13% [6].
At our knowledge, isolated intra abdominal localization with obstructive jaundice secondary to hepatic hilar lymph node compression in CLL has not been described.
We report the case of a 72 year-old man without past medical history, suffering from jaundice and pruritus for 1 month associated with pain in right hypochondrium without fever in a context of weight loss.
The physical examination showed jaundice, no tenderness of abdomen nor hepatomegaly or palpable mass or lymphadenopathy.
Laboratory tests showed following findings: hemoglobin value of 13 gr/dL, leukocyte count of 12500/μL, lymphocyte count of 4100/μL, platelet count of 255000/μL, sedimentation rate of 52 mm/hour, prothrombin rate of 63%, cholestasis; alkaline phosphatase of 578 IU/L(3.8 times the normal values), with high bilirubin levels (80μmol/L), moderate hypertransaminasemia: aspartate transaminase / alanine transaminase of 107 IU/L /132 IU/ L(normal value:40 IU/L). An active Hepatitis C virus (HCV) infection, genotype Ib, has been detected concomitantly. The viral load count was 3,159,150 IU/mL.
The abdominal ultrasound supplemented by an abdominopelvic computed tomography showed: a dilation of intrahepatic bile ducts, gall bladder and main bile duct in its pedicle portion (35 mm ) upstream of an inter hepatopancreatic lymph node mass of 53 mm x 40 mm. This mass comes into contact with common and proper hepatic artery with loss of safe fatty edging. The liver was of normal size, with regular contours and homogeneous enhancement as well as the pancreas (figures 1 and 2).
CT-guided biopsy of lymph node confirmed the diagnosis of CLL.The immunohistochemistry of the specimen indicated that the cells were positive for CD5, CD19, CD20, CD23 and CD4 but negative for CD10, Bcl-6 and Cyclin D1 (Figure 3).
Flow cytometry was typical of B-cell CLL being CD5-positive.
The computed tomography scan of neck, chest and pelvis showed no other deep lymphadenopathy.
Based on Rai and Binet CLL staging systems, patient’s disease was classified as stage I and stage A, respectively. Although, the early stage of disease, the strategy of ‘wait and see’ was not proposed because of symptomatic obstructive jaundice.
The patient had an endoscopic retrograde cholangiopancreatography with plastic biliary stent placement.Rituximab and Reduced Dose CHOP (cyclophosphamide, doxorubicine, vincristine and prednisone) (R-miniCHOP) protocol was established concomitantly with direct anti-viral drugs (DAAs) based on Sofosbuvir and Ledipasvir during 12 weeks. The patient was achieving sustained virological response.
After 6 cycles of chemotherapy for 6 months, restaging studies revealed disappearance of hepatic hilar lymphadenopathy in CT-scan, performed 12 months later (figure 4).
CLL is characterized by accumulation of functionally incompetent monoclonal lymphocytes which take origin in bone marrow and can spread and infiltrate organs of hemopoietic system, especially lymph nodes [7]. It mainly affects men in seventh decade and it is associated with a good prognostic; the 5-year survival rate is 86% [8]. Enlarged lymph nodes are the classical revealing manifestation, which can be unifocal, multifocal or affecting soft tissue as [9,10].
GI involvement in CLL is a manifestation uncommonly reported. The most important to mention are complications as liver failure, cholecystitis, peritonitis, pancreatico-biliary obstruction and thrombosis of surrounding mesenteric arteries and veins [11–13]. Jaundice is an extremely rare symptom in CLL. It has been reported in a rare cases associated with cholangiopathy as a consequence of lymphocytic infiltration within the biliary epithelium [14], with non specific canalicular stasis [15], with hepatomegaly due to Richter's transformation [16]. In pancreatic leukemia, Choi and al reported four patients with leukemic involvement of pancreas or biliary tract, presenting with symptoms of obstructive jaundice [17]. However, to our knowledge, involvement of hepatic hilar lymph nodes associated with obstructive jaundice revealing CLL, like in our patient, has not been described. Moreover, the association between LLC and HCV infection, in our patient, should be highlighted. HCV is a RNA virus which has a mainly hepatic tropism [18]. The capacity to replicate in lymphocytes sustains HCV infection association with occurrence of chronic lymphoproliferative disorders [19]. Along with, increased prevalence of HCV infection in some lymphoproliferative diseases has been recently reported, estimated at 1.4% regarding CLL [20]. Nevertheless, the exact mechanism linking HCV infection with lymphoproliferation is still unknown [21,22]. Interestingly, several studies suggested that increased expression of CD38 and CD23 has been found in CLL patients with viral coinfection and these markers are related more to disease progression risk [23]. Other findings shown that HCV does not induce disease progression risk, but a higher risk of resistance to treatment [24-25]. Our case is unique for several reasons; obstructive jaundice was an initial symptom yielded to positive diagnosis, CLL presented with isolated intra-abdominal lymphadenopathy despite early stage of disease and association with an active hepatitis C virus infection. The latter should be prominently mentioned as a factor that can be controlled by DAAs to improve chemotherapy response.
The present case is an example of a rare presentation of CLL. It is extremely important to keep in mind the possibility of leukemic infiltrate, in case of unexplained intra-abdominal mass, especially in patients with HCV infection, and to highlight the probability to have a complete response to chemotherapy associated with DAAs.
Written informed consent was obtained from the patient for publication of this case report and accompanying images.
The work was approved by the Ethics Committee of Mohamed Taher Maamouri hospital.
Authors thank Paramedical team of oncology and Gastroenterology department.
All authors contributed to this manuscript.
This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
 17.1 (1998): 28.
Citation: Laabidi S, et al. A Case of Chronic Lymphocytic Leukemia Revealed by an Obstructive Jaundice. Oncol Cancer Case Rep. 2022, 08 (02), 001-002
Received: 03-Mar-2022, Manuscript No. OCCRS-22-55269; Editor assigned: 05-Mar-2022, Pre QC No. OCCRS-22-55269(PQ); Reviewed: 18-Feb-2022, QC No. OCCRS-22-55269(Q); Revised: 20-Mar-2022, Manuscript No. OCCRS-22-55269(R); Published: 27-Mar-2022, DOI: 10.37532/occrs.22.8.(2).002
Copyright: 2022 Laabidi S, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.