Introduction
Chylothorax is the accumulation of chyle in the pleural area. While short and medium chain fatty acids are converted to free fatty acids by intestinal lipases and absorbed into the portal circulation, larger molecules are absorbed into the thoracic duct system in the form of chylomicrons together with the lower limb lymphatic drainage. Traumatic or non-traumatic damage of this lymphatic system results in the accumulation of chylous material in the pleural cavity. Traumatic damage is most frequently caused by surgery, while non-traumatic damage is most frequently caused by malignancies (1).
Waldenström’s macroglobulinemia is a disease in the B lymphoproliferative diseases group with immunoglobin M (IgM) monoclonality and the production of IgM heavy chain and light chain (2). The clinical presentation of the disease varies, including fever, weight loss-like constitutional symptoms, symptomatic anaemia, thrombocytopenia, amyloidosis and related organ involvement symptoms, hyperviscosity, lymphadenopathy, organomegaly, etc, it may present with different clinical manifestations. The cure expectancy for Waldenström’s macroglobulinaemia is still not expectable (2). In this case report, we present a patient with a clinic of Waldenström macroglobulinaemia who presented with an aggressive lymphoblastic lymphoma associated with an aggressive and refractory clinic of a chylothorax.
Case Report
A 52-year-old male patient applied to the general surgery outpatient clinic in June 2019 with complaints of weight loss and shortness of breath. Computed tomography (CT) results revealed a mass with a malignant soft tissue density, extending from the thoracic level along the paravertebral area to the inferior area, surrounding the aorta and its branches in the retroperitoneum and also surrounding the iliac arteries. In the upper abdomen, a mass lesion extending towards the portal hilar area and along the superior mesenteric artery axis was extended to the midline of the abdomen. In the thoracic images, a free fluid of up to 130 mm was found between the pleural leaves on both sides, and a view of atelectasis was observed at the level of the adjacent lower lobe segment.
The patient was referred to the haematology clinic after a biopsy of the abdominal mass and bone marrow was performed. Biopsy from the mass was reported to be compatible with a lymphoplasmocytic lymphoma in the presence of plasmoid differentiation B cell neoplasia and Waldenström clinic. Bone marrow biopsy showed a low-grade CD20 (+) lymphoid infiltration in the bone marrow and was reported to be in favour of Waldenström’s macroglobulinaemia involvement (Figure 1). In addition to anaemia and the sedimentation rate, the serum IgM level was 11 g/L and the free kappa level was 137 mg/L. Furthermore, total protein was 9.2 g/dL, albumin was 3.5 g/dL, globulin was measured as 5.7 g/dL and aBeta 2 microglobulin was 8.8 mg/L. Bilateral evacuating thoracentesis was performed because of the bilateral massive pleural effusion causing respiratory distress and desaturation (Figure 2). A catheter was inserted and the pleural effusion was evaluated to be compatible with a chylothorax (Figure 3). A nephrostomy catheter was inserted because of bilateral grade 3 hydronephrosis, predominantly on the left side due to compression from the abdominal mass.
Cyclophosphamide -adriamycin -vincristine -prednisolone (CHOP) regimen (cyclophosphamide 750 mg/m²/day intravenous (iv). 1st day, adriamycin 50 mg/m²/day iv.1st day, vincristine 1.4 mg/m² /day iv. 1st day (maximum 2 mg) prednisolone 100 mg/day iv (days 1, 2, 3, 4, 5) was used in the first cycle. Positron emission tomography (PET)/CT imaging, which was performed on the 15th day after the second cycle of chemotherapy, showed that the patient’s tumour was stable in size, but metabolic partial regression was observed. Bortezomib-dexamethasone-rituximab (BDR) regimen (bortezomib 1.3 mg/m2, dexamethasone 40 mg iv day 1, 4, 8 and 11; rituximab 375 mg/m2 iv day 11) was planned because of the therapy-resistant chylothorax originating from the thoracic tube. His mass was the only metabolic responsible and did not diminish in size. The patient had a dramatic clinical response with a complete regression of the chylothorax after the first cure treatment. Talc plorodesis was performed by pulling the chest tube, and the nephrostomy catheter was also removed. The patient was discharged to continue treatment as an outpatient. A written informed consent was obtained from our patient.
Discussion
Chylothorax with chyle in pleural area is a rare clinical presentation. It is also a rare cause of pleural effusion with chyle leakage into the pleural space. It has both traumatic and non-traumatic causes. Among surgical factors, thoracic surgical procedures are the most common traumatic factors (3,4). Non-traumatic causes are most frequently seen due to malignancies. Given that Waldenström’s macroglobulinaemia also has a very rare incidence with 0.38/100,000 (5), the clinical association between Waldenström’s macroglobulinaemia and chylothorax is very interesting.
The second important point to highlight in our case is the response to the preferred treatment regimen. For this case with Waldenström’s macroglobulinaemia, CHOP therapy was chosen as the preferred treatment since it does not contain rituximab, due to the risk of developing hyperviscosity in line with the recommendations of current treatment guidelines, followed by rituximab CHOP in the second cure. Due to the persistence of the pleural effusion and chylothorax, the patient was started on a BDR chemotherapy regimen recommended as the second-line chemotherapy treatment (6-8). We had a good clinical response with the BDR regimen, which is consistent with the literature. In the literature, similar rare cases presenting with chylothorax have been. In these case reports, it is noted that regimens based on bortezomib and its combinations are recommended.
Chylothorax is a very rare clinical presentation, and traumatic factors play a major role in its aetiology. Although malignancies lead to non-traumatic causes, Waldenström’s macroglobulinaemia is a rare cause. Standard treatment regimens vary, and responses to treatment also vary. Our case was resistant to the first line treatment, but showed a good clinical response with a bortezomib and rituximab-based treatment.
Ethics
Informed Consent: A written informed consent was obtained from our patient.
Peer-review: Internally peer-reviewed.
Authorship Contributions: Concept - İ.S., M.H.D., H.G., H.M., O.Y.; Design - İ.S., M.H.D., H.G., H.M.; Data Collection or Processing - İ.S., H.M.; Analysis or Interpretation - İ.S.; Literature Search - İ.S., H.M.; Writing - İ.S., M.H.D., H.G., H.M., O.Y.
Conflict of Interest: No conflict of interest was declared by the authors.
Financial Disclosure: The authors declared that this study received no financial support.