Chyloperitoneum, or chylous ascites, is a rare condition characterized by milky-appearing fluid with elevated triglyceride content and the presence of chylomicrons. Malignancy, specifically lymphoma, is reported to be the predominant cause in Western countries. Previously, the prognosis for patients with chyloperitoneum due to lymphoma has been reported as poor. We present a case of chyloperitoneum and chylothorax due to follicular lymphoma with excellent response to bendamustine and Rituxan. A review of the literature indicates that patients with chyloperitoneum associated with lymphoma generally have a favorable response to contemporary treatment regimens.
Chyloperitoneum, also known as chylous ascites, is a rare condition characterized by milky-appearing lymph fluid in the abdominal cavity. It presents slowly with gradual, painless abdominal distension. The patient may experience weight gain and dyspnea as the fluid exerts pressure on the diaphragm. Paracentesis is necessary for diagnosis as it will reveal turbid, milky fluid that is distinguished by chylomicrons and an elevated triglyceride content (>200 mg/dL). Abdominal malignancies, specifically lymphoma, is reported to be the predominant cause in Western countries [
In 1982, Press et al. reviewed the largest series of patients with chyloperitoneum due to lymphoma and found prognosis to be poor—only 2 of 13 patients experienced long-term survival (greater than three months) [
A 78-year-old woman presented to an outside facility with dyspnea and abdominal fullness. Physical examination revealed abdominal distension with a fluid wave and decreased breath sounds with dullness to percussion in the left hemithorax. Paracentesis and thoracentesis were both performed, revealing creamy opaque fluid with an elevated triglyceride content. Cytological examination of the peritoneal fluid showed chylomicrons. Prior to presentation to our institution, she underwent thoracentesis four times and paracentesis five times with rapid recurrence of symptoms.
On presentation to our institution, computed tomography (CT) scan of the abdomen and pelvis revealed para-aortic lymphadenopathy (Figure
Coronal and transverse CT imaging of para-aortic lymphadenopathy.
Lymph node biopsy pathology displaying small lymphoid cell proliferation.
Based on the histologic appearance and the low expression of Ki-67, the patient was diagnosed with low-grade follicular lymphoma. Due to the bulkiness of her disease with the multiple recurrent effusions, chemotherapy was pursued. Further staging workup with bone marrow biopsy and PET scan was deferred since it would not have changed management with the plan to pursue systemic therapy, regardless. She received four cycles of bendamustine and rituximab with complete resolution of both the ascites and pleural effusion. At one-year follow-up since complete resolution of her disease, she remained asymptomatic without reoccurrence of effusions; therefore, further chemotherapy was not pursued.
Serous effusions are relatively common among malignant lymphomas. The most common effusion site is pleural, affecting 20–30% of NHL and Hodgkin lymphoma (HL), followed by pericardial and peritoneal [
Targeting the underlying cause of chylous ascites is crucial to resolution, as in our patient who received follicular lymphoma-directed therapy. Treatment preferences for follicular lymphoma are varied since patients can live well for many years without treatment. Generally, stage I and stage II disease can be treated with watchful waiting or radiotherapy, whereas bulky stage II or stage III-IV can be treated with rituximab plus other biologic agents or chemotherapy, with or without radiotherapy [
To evaluate the current state of chyloperitoneum prognosis and treatment secondary to lymphoma, a search of the MED-LINE database was performed using the search terms “chylous ascites” or “chyloperitoneum” and “lymphoma”. Fourteen cases from 10 unique publications were found that described chylous ascites secondary to lymphoma [
Chyloperitoneum associated with lymphoma.
Case number | Author/year | Age/sex | Site | Diagnosis | Treatment | Outcome |
---|---|---|---|---|---|---|
1 | Current case | 78/F | Peritoneal, |
Follicular lymphoma | Bendamustine + rituximab | Resolution |
2 | Arasawa, 2014 [ |
74/M | Peritoneal | DLBCL | R-CHOP | Resolution |
3 | Jiang, 2013 [ |
28/M | Peritoneal, |
Non-Hodgkin Lymphoma | DOLP + |
Resolution |
4 | Yamamoto, 2013 [ |
87/M | Peritoneal | DLBCL | R-CHOP | Resolution |
5 | Etonyeaku, 2012 [ |
18/F | Peritoneal, |
Burkitt’s lymphoma | MEV | Died due to treatment effects |
6 | Kashyap, 2011 [ |
21/F | Peritoneal, |
DLBCL | R-CHOP | Resolution |
7 | Ionnidou-Papagiannaki 2009 [ |
38/M | Peritoneal, |
T-cell lymphoma | CHOEP | Died due to GI bleeding |
8 | Gonen, 2007 [ |
53/M | Peritoneal | Hodgkin lymphoma | ABVD | Regression after two cycles chemotherapy |
9 | Ward, 2008 [ |
Middle age/F | Peritoneal | Follicular lymphoma | Chemotherapy unspecified | Resolution |
10 | Bachmeyer, 2004 [ |
71/M | Peritoneal | Follicular |
CHVP + interferon alpha | 50% reduction |
11 | Oosterbosch, 1995 [ |
68/F | Peritoneal, |
DLBCL | CVP | Regression |
12 | Hufford, 1988 [ |
49/F | Peritoneal | Mixed cellularity (Hodgkin lymphoma) | CVP, doxorubicin |
Resolution |
13 | Hufford. 1988 [ |
67/F | Peritoneal | Small cleaved cell (NHL) | CVP | No response to chemotherapy, resolution after diuretics |
14 | Hufford, 1988 [ |
58/M | Peritoneal | Hodgkin lymphoma | MOPP | Resolution |
15 | Hufford, 1988 [ |
60/M | Peritoneal | Small cleaved cell (NHL) | CHOMP + bleomycin | Resolution |
R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone; CVP: cyclophosphamide, vincristine, and prednisolone; CHOMP: cyclophosphamide, doxorubicin, vincristine, and prednisone + bleomycin; MOPP: Mustargen, Oncovin, procarbazine, and prednisone; CHVP: cyclophosphamide, doxorubicin, etoposide, and prednisolone; ABVD: doxorubicin, bleomycin, vinblastine, and dacarbazine; CHOEP: cyclophosphamide, hydroxydaunorubicin, Oncovin, etoposide, and prednisone; DOLP: daunorubicin, vincristine, L-asparaginase, and prednisone; HDAra-C: high dose cytarabine; MEV: methotrexate, cyclophosphamide, and vincristine.
Our review of the contemporary literature suggests that the advent of superior chemotherapy regimens has led to marked improvement in survival and resolution of symptoms in most patients. Prior to the development of these regimens, lymphoma-related chylous effusions carried a dismal prognosis with greater than 90% of patients dying within three months of diagnosis before 1982 [
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
The authors declare that they have no competing interests.
Brice Taylor conceived of the study and drafted the paper. Stephanie Parks Taylor acquired patient information, participated in the concept of the report, and helped to draft the paper. Megan Jagosky helped with the literature review and correspondence. All authors read and approved the final paper.