1. INTERSTING CASE OF ASCITES PROF. DR.P.VIJAYARAGHAVAN’S UNIT DR.C.R.RAJKUMAR

2. 63yrs old male admitted with C/O Abdominal distension - 1 month Bilateral leg swelling -15 days

3. History of present illness: Abdominal distension -1month duration, -slowly progressive, uniform Bilateral legs swelling – 15 days duration, -insidious, progressive Shortness of breath – 15 days Abdominal pain – past few days -diffuse, dull aching Decreased urine output – past few days

4. No H/O jaundice, hematemesis, melena No H/O Loose stools No H/O fever H/O Loss of appetite + H/O Loss of weight +

5. PAST HISTORY H/O jaundice present - 6yrs back -details not known Not a known SHT / DM / PT

6. PERSONAL HISTORY Mixed diet Known chronic alcoholic ->10yrs -180 – 270ml /105 gm / day Chronic smoker

7. EXAMINATION General examination Conscious Oriented Afebrile Dyspneic Mild pedal edema Pallor+ An icteric No clubbing No cyanosis Few lymph-nodes in left cervical region 0.5 to 1cm in size.

8. SYSTEMIC EXAMINATION CVS – S1, S2 Heard no murmurs RS – NVBS Heard no added sounds P/A –Soft distended firm to hard liver palpable irregular surface free fluid + fluid thrill+, few dilated veins  flow below upwards CNS –NFND

9. IMPRESSION Ethanol related DCLD with ascites ? Malignant transformation

10. INVESTIGATIONS CBC Hb -9 gm / dl TC - 5400 DC - P-60 L-30 E-10 ESR - 10 / 22mm MCV - 88 fl MCH – 29pg RBC – 5000 millions PLATELETS – 80000

11. RFT Blood sugar - 81 mg/dl Urea - 30mg /dl Creatinine - 0.9mg /dl

12. USG ABDOMEN Liver – 9.5 cm , altered texture , nodular surface Free fluid + GB – Apparent GB wall thickening+ Pancreas – 10 * 9 cm hetero echoic lesion in the para aortic area displacing the pancreas Spleen – 11.7 cm Kidney – RT -10* 5.3 cm, left 9.9 * 4.5 cm increased echo , 1.8 * 2 cm cystic lesion in the upper pole of RT kidney multiple lesion in the LT kidney IMPRESSION Parenchymal liver disease with ascites. Para aortic lymphadenopathy+ Bilateral renal cortical cyst.

13. ASCITIC FLUID ANALYSIS Appearance – milky (chylous) Total WBCs – 3000 cells/cumm Neutrophils – 6-8 / hpf Sugar – 87 mg/dl Total protein 2.9 gm /dl Albumin 1.4 gm / dl SAAG1.2

14. Amylase - 271 u/ l Cytology– plenty of lymphocytes along with reactive mesothelial cells on eosinophilic backgrounds Adenosine deaminase - 42.4 u/l Ascitic fluid TGL: 210mg%

15. SP 3 Hb -8.3 gm /dl TC - 6900cells/cumm DC - P-63 , L-26 , E -11 Platelets – 16000 PCV -34.8 MCV - 91.9 MCH - 29.6 MCHC- 32,5 ESR - 38 mm/hr PT -14 sec INR - 1 APTT - 26

16. Blood sugar 81mg/dlUrea 26.16 mg/dlCreatinine -1.12 mg/dl Total bilirubin -0.52 mg/dl Direct - 0.2mg/dl AST - 47u/l ALT - 25 U GGT - 30 U SAP -249 Total protein -6.3 g/dl Albumin -2.6 /dl Globulin - 3.7 g/dl Na+ -131.7 meq/l k+ - 4.33 meq/l Cl- 98.4meq/l

17. SEROLOGY HIV Antibody - negative HBSAg - negative Anti HCV - negative URINE complete analysis Colour - yellowish Appearance - turbid PH -6.0 Specific gravity -1.030 Albumin + Sugar , blood , ketone - negative Nitrite - negative Bilirubin + Urobilinogen - +

18. Urine microscopy leucocytes 5-6 cells/hpf EP cells – 2-3/ hpf

19. Chest physician opinion: - RT pleural effusion OGD SCOPY: -Erosive gastritis - No esophageal varices

20. Pleural fluid analysis: Sugar – 93 Protein – 2.8

21. With the above investigations what are the possibilities? Comment Causes of ChylousAscites:

22. Points favouring TB ↑ ascitic fluid ADA ↑ proteins  exudate Right pleural effusion Para-aortic nodes Points against – High SAAG How to proceed?

23. CT abdomen Multiple para-aortic nodes+. Ascites with right pleural effusion. CT being inconclusive, what to do next??

24. The small cervical nodes started becoming more conspicuous FNAC -CERVICAL LYMPH NODE possibility of chronic lymphoid leukemia / small lymphocytic lymphoma

25. INGUINAL NODE BIOBSY Diffuse effacement of lymphnode architecture by monotonous population of neoplastic cells [lymphocytes]. The cells are small with scant cytoplasm noncleved centrally placed nucleus with open chromatin. The neoplastic cells are seen to infiltrate the adjacent perinodal pad of fat. Blood vessels are interspread with in neoplastic cells. Impression Non Hodgkin lymphoma/ chronic lymphoid leukemia / small lymphocytic lymphoma

26. Patient had trouble with crossmatching  possible Auto Immune Hemolytic Anemia

27. Hematology opinion Suggested LDH Hematocrit To do Direct Coomb’s Test  to r/o AIHA

28. Plan to start chemotherapy after blood investigation But before we could start chemotherapy pt deteriorated and died of cardio respiratory arrest

29. Final diagnosis Small cell lymphoma infiltrating the liver causing portal hypertension and chylous ascites with possibly autoimmune hemolytic anemia.

30. CHYLOUS ASCITES Chylousascites is an uncommon clinical condition that occurs as a result of disruption of the abdominal lymphatics.

31. CAUSES Abdominal surgery Abdominal trauma Malignant neoplasms hepatoma, small cell lymphoma, retro peritoneal lymphoma Spontaneous bacterial peritonitis [SBP] Cirrhosis – upto 1% Peritoneal dialysis Abdominal tuberculosis Carcinoid syndrome Congenital defects of lacteal formation

32. classification True chylous ascites: fluid with high triglyceride content (>200mg%) Chyliform ascites: fluid with a lecithin- globulin complex due to fatty degeneration of cells. Pseudo chylous ascites: fluid that is milky appearance due to the pus

33. Chylousascites diagnosed by -The ascitic fluid triglyceride level is elevated in all pts with chylous ascites. -TGL >110 mg/dl, >200 definite -Elevated ascites : plasma triglyceride ratio [between 2.1 and 8.1] Complication Sepsis Sudden death

34. TREATMENT Chylous ascites is a manifestation rather then a disease depends on the treatment of the underlying disease or cause Supportive measures can relieve the symptoms such as repeated paracentesis, diuretic therapy, salt and water restriction Low-fat diet with medium chain triglyceride Octreotide is most likely effective

35. Non-hodgkin's Lymphoma of the Liver Lymphomatous infiltration of the liver is more common in non-Hodgkin's lymphoma (NHL) than in Hodgkin's disease. Hepatic involvement with NHL has been described in liver biopsy specimens in up to 53% of cases (and an even higher percentage at autopsy), is more common with small-cell varieties than with large- cell types.

36. Liver biochemical test results may be abnormal (primarily an increase in serum alkaline phosphatase), and hepatomegaly may be detected. Rarely, NHL can present as a primary hepatic lymphoma. Apart from human immunodeficiency virus–associated lymphomas, primary hepatic lymphoma has a better prognosis than NHL because of the possibility of cure with successful resection.

37. Jaundice is rare in patients with NHL and in the past was considered a terminal occurrence. Jaundice secondary to extrahepatic biliary obstruction is more common in NHL (1.2%) than in Hodgkin's disease (0.3%), and biliary obstruction occurs most commonly at the porta hepatis, although primary lymphomatous involvement of the bile ducts has been reported in rare cases. In patients with NHL and jaundice, gallstones and pancreatic adenocarcinoma must be excluded.

38. Treatment Hepatic resection/Orthotopic liver transplant for primary hepatic NHL. Chemotherapy for CLL/SLL with secondary hepatic involvement: Fludarabine/cladribine is usually combined with an alkylating agent such as cyclophosphamide. Alemtuzumab – a monoclonal antibody against CD52 is tried in refractory cases.

39. Thank you