Refractory chylous ascites revealing follicular lymphoma: A case report

Abstract

Introduction and importance

Chylous ascites is an uncommon form of ascites characterized by milky fluid rich in triglycerides. It is associated with poor lymphatic drainage. We report a case of chylous ascites revealing a follicular lymphoma.

Case presentation

A 73-year-old man presented with a 6-month history of abdominal distension attributed to a chylous ascitis. The thoraco-abdomino-pelvic CT scan revealed voluminous intra- and retroperitoneal mass inseparable from the duodeno-pancreatic block and encompassing the mesenteric vessels, inferior vena cava and renal vessels; abundant ascites and multiple mediastinal, coeliomesenteric, retroperitoneal, iliac and inguinal adenomegalia. The diagnosis of follicular lymphoma was retained through a radio-guided biopsy of the retroperitoneal mass. The patient had weekly paracentesis and immuno-chemotherapy. The course was unfavorable, marked by infection of the ascites fluid after two cycles of immuno-chemotherapy. Our patient developed severe sepsis and died.

Clinical discussion

Chylous ascites in conjunction with follicular lymphoma is an exceptional presentation. The pathophysiological mechanism is an impediment to subdiaphragmatic lymphatic drainage caused by external pressure, leading to leakage of dilated subserosal lymphatic ducts into the peritoneal cavity. Histological confirmation is fundamental to manage chylous ascites resulting from lymphomas.

Conclusion

Chylous ascites revealing lymphoma is a unique condition. The key to management is the treatment of the underlying etiology.

Highlights

• •Rare Presentation of Follicular Lymphoma: The manuscript highlights the rarity of chylous ascites as an initial manifestation of follicular lymphoma, emphasizing the unique and atypical nature of this presentation.
• •Challenges in Management: It emphasizes the necessity for iterative paracentesis, potential complications such as severe sepsis, and the importance of histological confirmation through biopsy for appropriate management.
• •Treatment Strategies and Prognosis: The manuscript concludes by underscoring the uniqueness of chylous ascites as a revealing sign of lymphoma and emphasizes the critical role of recognizing atypical presentations for effective management.

1. Introduction

Chylous ascites is a rare form of ascites characterized by milky fluid rich in triglycerides. It is associated with the disruption of the lymphatic system, either due to an obstruction or post-traumatic injury [1].

Chylous ascites in conjunction with follicular lymphoma is an exceptional presentation.

We report the case of a patient in whom chylous ascites served as initial manifestation of follicular lymphoma.

This work was reported in line with the SCARE criteria [10].

2. Observation: a case study

A 73-year-old man presented to surgery department with a 6-month history of abdominal distension and peripheral edema.

His medical history was positive for diabetes and hypertension, with no history of alcohol or tobacco use.

On physical examination, the patient was neurologically and hemodynamically stable.

He was mildly polypneic with no respiratory distress. He had no fever or jaundice.

He had no signs of right heart failure and no cervical or axillary adenopathies.

Abdominal examination revealed abundant ascites without collateral venous circulation or hepatosplenomegaly and poorly circumscribed epigastric mass. Furthermore, bilateral centimetric inguinal adenopathy, hydrocele and edema of the lower limbs guarding the bucket were noted.

The haemogram showed haemoglobin at 13.6 g/dl, 7690 leukocytes per mm3 with 63 % neutrophils and 30 % lymphocytes, and 219,000 platelets per mm3.

Liver tests and creatinine levels were normal. Albumin level was 34.4 g/l and haemostasis test was normal. HbS antigen, hepatitis C and HIV serologies were negative.

Ascites puncture was performed to prevent eventual respiratory distress. It revealed a milky fluid (Fig. 1), with an albumin of 10 g/dl and a serum-ascites albumin gradient of 24.4 g/dl. The ascitic fluid contained 190 cells per mm3 (80 % lymphocytes) and 3.3 g/l of triglycerides while serum triglycerides level in serum was 1.2 g/l. No malignant cells were detected cytological examination of ascites fluid.

Fig. 1.

Milky-appearing ascitic fluid following ascites puncture.

A thoraco-abdomino-pelvic CT scan was performed to complete the etiological assessment. It revealed a voluminous intra- and retroperitoneal tissue mass, with lobulated contours and heterogeneous enhancement, measuring 19 cm in length, inseparable from the duodeno-pancreatic block and encompassing the mesenteric vessels, inferior vena cava and renal vessels (Fig. 2). There was abundant ascites, nodular infiltration of the greater omentum, and multiple mediastinal, coeliomesenteric, retroperitoneal, iliac and bilateral inguinal adenomegalia. The liver displayed normal volume and homogeneous enhancement.

Fig. 2.

Axial (A) and sagittal (B) sections of injected abdominal CT scan showing an intra- and retroperitoneal mass (↔) inseparable from the duodeno-pancreatic block and encasing the mesenteric vessels, inferior vena cava and renal vessels.

The level of lactate dehydrogenase (LDH) was 382 IU/l (normal <246).

CT scan findings, combined with elevated LDH levels, suggested lymphomatous pathology. A lymph node excisional biopsy was performed. However, histological examination revealed no malignant proliferation.

So we proceeded to a CT scan-guided biopsy of the retroperitoneal mass (posterior path). The histological examination revealed a nodular infiltration of small lymphocytes. The immunohistochemical analysis showed that these cells expressed CD19, CD20, CD10, bcl2, and bcl6, but did not express CD5 and CD43; Ki67 proliferation index was <10 %. The diagnosis of follicular lymphoma, grade 3A, has been retained.

Weekly evacuation of ascites was necessary. Immuno-chemotherapy using the RCHOP protocol (Rituximab, cyclophosphamide, doxorubicin, oncovin and prednisone) was started.

The course was unfavorable, marked by infection of the ascites fluid after two cycles of immuno-chemotherapy. Our patient developed severe sepsis and died.

3. Discussion

Chylous ascites is a rare form of ascites characterized by milky fluid with triglyceride levels above 200 mg/dl. It occurs when the lymphatic channels are obstructed or damaged, often as a result of conditions such as lymphomas, infections like tuberculosis and lymphatic filariasis, inflammatory diseases, or abdominal trauma [2].

In developing countries, the most common causes of chylous ascites are infections. In developed countries, the main causes are abdominal neoplasia and cirrhosis. As far as neoplasia is concerned, lymphoma is implicated in almost 10 % of cases [1,2].

Follicular lymphoma is the most common subtype of non-Hodgkin's lymphoma, and tends to have an indolent course. It accounts for over 25 % of all non-Hodgkin's lymphomas.

Chylous ascites is an exceptional manifestation of lymphomas. The pathophysiological mechanism, in this case, is an impediment to subdiaphragmatic lymphatic drainage caused by external pressure, leading to leakage of dilated subserosal lymphatic ducts into the peritoneal cavity [3].

Rapid onset of chylous ascites may be responsible for abdominal compartment syndrome, which can be critical [4]. In our case, the ascites developed progressively, and our patient did not present such a complication.

Chylous ascites is associated with electrolyte and protein depletion, predisposing to infections [5]. Infection of ascites fluid in a fragile patient could lead to severe sepsis, as in our case.

Iterative paracentesis is often necessary in cases of recurrent ascites. The aim is to improve symptoms and quality of life [6]. A high-protein and low-fat diet with medium-chain triglycerides is also recommended [3]. Dietary measures were ineffective in our case.

Histological confirmation through h radioguided or surgical biopsy is fundamental to manage chylous ascites resulting from lymphomas. The key to management of chylous ascites is the treatment of the underlying etiology [3].

It is based on radiotherapy, immunotherapy and chemotherapy, which can be combined.

The R-CHOP protocol combining Rituximab with CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone) is associated with a 5-year survival of 94 %. Intraperitoneal drug administration offers better results than the intravenous route [7].

Radiotherapy is a crucial therapeutic modality in the treatment of follicular lymphoma. It is associated with complete remission in 60 % of cases, and allows long-term local control [8]. Low-dose radiotherapy of 4 Gy delivered in 2 fractions of 2 Gy each is a highly effective choice for advanced or refractory indolent lymphoma, exhibiting elevated and enduring response rates [9].

Failure to treat lymphoma is an indication for thoracic duct ligation or peritoneoveinous shunting to control recurrent chylous ascites [3].

4. Conclusion

Chylous ascites revealing lymphoma is a unique condition. This case underscores the importance of recognizing atypical presentations of lymphomas and the challenges associated with their management.

The key to management of chylous ascites is the treatment of the underlying etiology.

Ethical approval

Not applicable. Our institution requires no ethical approval for case reports.

Funding

Not applicable.

All authors read and approved the final manuscript.

Author contribution

Yassine Tlili: conception and design, acquisition of data, drafting the article, revising it critically for important intellectual content, final approval of the version to be published

Zied Hadrich: conception and design, acquisition of data, drafting the article, revising it critically for important intellectual content, final approval of the version to be published

Montacer Hafsi: conception and design, acquisition of data, drafting the article, revising it critically for important intellectual content, final approval of the version to be published

Sahir Omrani: final approval of the version to be published

Rached Bayar: final approval of the version to be published

Hafedh Mestiri: final approval of the version to be published

Guarantor

The corresponding author is the guarantor of submission.

Research registration number

None

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Availability of supporting data

Not applicable.

Conflict of interest statement

All authors declare that they have no conflicts of interest.