|Year : 2014 | Volume
| Issue : 2 | Page : 184-186
Hodgkin's lymphoma presenting as chylothorax
Sanjeev Kumar Verma1, Saurabh Karmakar2
1 Department of Pulmonary Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India
2 Department of Pulmonary Medicine, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh, India
|Date of Web Publication||1-Apr-2014|
Department of Pulmonary Medicine, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Verma SK, Karmakar S. Hodgkin's lymphoma presenting as chylothorax. Lung India 2014;31:184-6
A patient presented with pleural effusion, which on aspiration revealed chylothorax. The patient also had generalized lymphadenopathy. Histopathology of accessible lymph node revealed nodular sclerosis type Hodgkin's lymphoma.
A 35-year-old, nonsmoker female presented to us with chief complaints of loss of appetite and loss of weight for a year and half, off and on breathlessness, and high-grade fever for 6 months. Her treatment history from perusal of records revealed that she had undergone pleurocentesis at a previous center. Fine needle aspiration cytology (FNAC) of cervical lymph nodes had been inconclusive. Over the past 1 year, she had been prescribed 6 months of daily regimen Anti Tuberculosis Therapy, which she completed, followed by retreatment regimen. She did not get any relief and attended our center.
Her general examination was unremarkable, except for pallor and bilateral cervical swellings. The cervical swellings were in the lower cervical region, in the deep cervical lymph node group and were firm, discrete rubbery in consistency, and nontender; the largest being 2 × 2 cm. Her breast, thyroid, and gynecological evaluation were normal. She had negative enzyme-linked immunosorbent assay (ELISA) test for human immunodeficiency virus (HIV) and was nondiabetic. Her abdominal examination revealed significant hepatomegaly of three fingers. Splenomegaly was absent and the rest of the quadrants were soft and nontender.
Pleural effusion was detected sonologically and aspiration from right hemithorax revealed fluid of milky appearance. The biochemical analysis was as follows: Protein 4.58 g/dl, sugar 80 mg%, total leukocyte count (TLC) 1,000/mm 2 , differential leukocyte count (DLC) - lymphocytes 80%, polymorph 20%, cholesterol 43 g%, and triglyceride 690 mg%. Pleural fluid was negative for acid-fast bacilli (AFB) and gram stain on smear and sterile on culture.
A contrast-enhanced computed tomography (CECT) thorax showed lymph node masses in the anterior mediastinum in the upper and lower pre- and paratracheal stations [Figure 1]. The lymph node masses were encasing the major vessels and compressing the trachea. There was collapse and consolidation of the right upper lobe with consolidation and small cavitary lesion in the anterior segment of the left upper lobe with large right-sided pleural effusion and large mediastinal and right axillary lymphadenopathy. Her CECT abdomen revealed significantly enlarged discrete and confluent lymph nodes in peripancreatic, periportal, celiac, para-aortic, aortocaval, paracaval, and mesenteric regions [Figure 2]. Fiber-optic bronchoscopy was done. Endobronchial appearance was within normal limits and bronchoalveolar lavage was collected from both upper lobes, which was negative for Acid Fast Bacilli on smear. Gram aerobic, fungal, and Mycobacterium tuberculosis (MTB) cultures were sent and were reported sterile.
|Figure 1: Computed tomography (CT) thorax showing mediastinal lymphadenopathy|
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Since her past FNAC was inconclusive and we suspected malignancy, an excision biopsy of right cervical lymph node was done. Histopathological analysis of the sections revealed nodular sclerosis type of Hodgkin's Lymphoma [Figure 3]. The paraffin sections were positive for CD 30, CD15, and Epstein-Barr virus latent membrane protein (EBV LMP1) on immunohistochemical staining of the Reed-Sternberg cells More Details. We diagnosed it as a case of nodular sclerosis type Hodgkin's lymphoma Ann Arbor stage III EB. We referred the patient to the department of Hematology for chemotherapy. However, she was lost to follow-up.
Chylothorax was first described by Bartolet in 1633 and by 1944, only 105 cases had been recorded.  Chylothorax is the presence of chyle in the pleural space, secondary to leakage from the thoracic duct or one of its main tributaries. Chyle (from the Latin word chylus, or juice) consists of chylomicrons and very low density lipoproteins absorbed from the small intestine, secreted into intestinal lymphatics, and moved into cisterna chyli and then to thoracic duct. Chyle can accumulate in either the left or right pleural space, depending on where the thoracic duct is disrupted. If the disruption occurs below the fifth thoracic vertebra, then right-sided chylothorax occurs; and when above the fifth thoracic vertebra, then chylothorax is left-sided.  It is usually right-sided, since most of the duct is within the right hemithorax. 
The best way to establish the diagnosis of chylothorax is by measuring the triglyceride and cholesterol levels of pleural fluid. If the pleural fluid triglyceride level is above 110 mg/dl and the ratio of pleural fluid to serum cholesterol is <1.0, then pleural effusion is diagnosed as chylothorax.  In our patient, the pleural fluid triglyceride level was 690 mg/dl and the pleural fluid to serum cholesterol ratio was 0.26 (<1). The demonstration of chylomicrons in the pleural fluid by lipoprotein analysis establishes the diagnosis of chylothorax. 
The most common symptoms of chylothorax include dyspnea and orthopnea. Chyle is not irritating to the pleura, so chest pain is rare. Chyle contains T lymphocytes, fat-soluble vitamins and a significant amount of the dietary calories from fat intake. The loss of these vitamins, calories, and lymphocytes from the body through chyle leads to malnourished and immunodeficiency state. Chyle is bacteriostatic, and thus chylothorax is rarely complicated by empyema. Severe metabolic acidosis and hypoprothrombinemia can also occur.  The underlying condition should be treated prior to a conservative or surgical approach for management of chylothorax.  Conservative methods include administration of medium chain triglycerides, somatostatin, or octreotide; pleurodesis; and lymphangiography followed by embolization. Lymphoma-related chylothorax that persists after chemotherapy/radiotherapy is best managed by chemical pleurodesis, as majority of patients are unfit for surgical closure of chyle leak.  Surgical methods include ligation of thoracic duct by thoracotomy or thoracoscopy and pleurectomy. Due to the rarity of chylothorax in Hodgkin's lymphoma, there is lack of data on effectiveness of each of the therapeutic options. 
In a case series of 203 patients over 21 year period, surgery and trauma followed by malignancy were the common causes of chylothorax. Lymphoma is the most common malignancy and non-Hodgkin's lymphoma is the usual type reported to cause chylothorax. Hodgkin's lymphoma, without any specification of subtype is responsible for only 1.4% of chylothoraces. 
Nodular sclerosis type of Hodgkin's lymphoma presenting with chylothorax has never been known in Indian literature, so we are reporting this case. Our case also reaffirms the role of CECT of thorax and abdomen for rapid noninvasive assessment of both compartments and an excision biopsy of peripheral lymph nodes; as workup needed to achieve diagnosis when lymphoma is suspected in a case of nontraumatic chylothorax. 
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[Figure 1], [Figure 2], [Figure 3]