ABSTRACT

Polyserositis is defined as general inflammation of serous membranes associated with simultaneous effusions in various cavities. This rare syndrome has some unusual etiologies some of which are end stage diseases. Tuberculosis, rheumatism, SLE are the usual causes, While haematological malignancies are on the other end of the spectrum. We came across one such case of polyserositis masquerading as tuberculosis which turned out to be Non-Hodgkin’s lymphoma. We present it here because of its rarity and the associated high mortality.

KEYWORDS

Polyserositis, Hypothyroidism, Non-Hodgkin’s lymphoma, Adenosine Deaminase, Concato’s disease, Angioimmunoblastic T cell lymphoma.

CASE REPORT

A 30 year old female presented with weight loss of 7 kgs over 4 months, high grade intermittent fever with chills and rigor for 15 days with night sweats. Since 1 week she became breathless progressing from Grade II to IV and is orthopneic. She has diffuse, dull and non-colicky abdominal pain since 4 days along with nausea and loss of appetite since 4 months. She was apparently normal before 4 months. She is not under treatment for any chronic illness. She is amenorrheic since 4 months. On examination she was conscious and febrile(1040 F) with pulse rate – 112/minute, BP – 110/70 mmHg, respiratory rate – 21/min, SpO2 – 95% with oxygen. She was thin built and pale. She had bilateral cervical lymphadenopathy with matting of right supraclavicular lymph nodes. The largest node was of 2×2 cm size, immobile with overlying normal skin. There was no sinus or scar. She had a palpable left axillary lymph node in the central group of size 4×4 cm, hard and mobile. Her cardiovascular and CNS examination was normal. Abdomen was diffusely tender to palpation. She had features of bilateral pleural effusion on clinical examination. We suspected tuberculosis and proceeded with further investigations.

Chest-ray revealed bilateral pleural effusion left more than right with mediastinal widening(Figure 1). From the blood investigations anemia with reactive thrombocytis and hypothyroidism was made out. Liver function was deranged but not suggestive of hemolysis. Peripheral smear revealed no more than anemia. Other blood investigations for the fever and weight loss were negative. Pregnancy and HIV were ruled out. Pleural fluid analysis showed an exudative lymphocytic effusion with very high ADA levels. Matted cervical lymphadenopathy with the haematological and pleural fluid analysis lead us to make a provisional diagnosis of extrapulmonary tuberculosis.

Before starting anti-tubercular treatment we did an ultrasonogram of the abdomen, which showed ascites with bilateral pleural effusion. Therapeutic thoracentesis was done and 800ml of hemorrhagic pleural fluid was drained. Oxygen therapy, diuretics, broad spectrum antibiotics and antipyretics were given. But the patient worsened so we did an echocardiogram which revealed mild pericardial effusion. We revised our diagnosis to Polyserositis and did further workup. CECT abdomen revealed left paraaortic lymphadenopathy, ascites and bilateral pleural effusion. Since the patient became very dyspneic CT chest couldn’t be done. FNAC of the cervical lymphnode was proceeded and it showed monotonous population of lymphocytes and few atypical lymphocytes in a background of RBCs and fibrinous material. It suggested Lymphoproliferative disorder of Non Hodgkins lymphoma type.

Excision biopsy of the right supraclavicular lymph node(Figure2) showed small to intermediate sized lymphoid cells infiltrating the adjoining perinodal fat, with predominant areas of lymph node showing infarction. Immunohistochemistry of the biopsy material showed CD20 negative and CD3 strong positivity in 90% of lymphoid cells suggestive of Non-Hodgkin’s Lymphoma of T cell lineage. By this time the patient’s vital organs started to drown in her own fluids. A final diagnosis of Polyserositis due to Non-Hodgkin’s lymphoma of T cell type was made. Further typing of the T cell variant couldn’t be done because of her unwillingness. Knowing the worse prognosis of her condition, her husband took her home and the next day she had expired.

DISCUSSION 

Concato’s disease is defined as progressive malignant polyserositis with large effusions of pericardium, pleura and peritoneum⁽¹⁾. 6 to 50% of non-Hodgkin’s lymphoma(NHL) present as pleural effusion of which 20% are chylothoraces, while 7 to 21% of Hodgkin’s lymphoma present with pleural effusion and 3% of are chylothoraces. In NHL 20 to 70% have evidence of mediastinal disease and 90% have disease elsewhere. Pleural effusion is frequently associated with large cell NHL compared to small cell variants⁽²⁾. Nodular sclerosis is the predominant Hodgkin’s variant in this setting. In NHL, patient’s survival is not adversely affected by the presence of pleural effusion as a presenting feature⁽³⁾. 40% of  Angioimmunoblastic T-cell lymphoma(AITL)  has pleural effusion. It extensively infiltrates the lymph nodes with atypical lymphocytes, proliferation of arborizing small vessels and amorphous acidophilic material deposits⁽⁴⁾. Atypical lymphocytes are also present. Most of AILD cases have monoclonal T-cell population and 95% have Epstein-Barr Virus infected cells. AILD presents with generalized lymphadenopathy, hepatosplenomegaly, rash, effusions and polyarthritis. Most of them have elevated ESR and LDH, hypergammaglobulinemia and anemia, thrombocytopenia and lymphopenia. It is generally an aggressive disease. Most of the treatment regimens include a combination of an alkylating agent and an anthracycline.

Tuberculous effusions are unilateral usually and have glucose more than 60mg/dl. Our patient had bilateral effusion with a 20mg/dl of glucose. Adenosine deaminase levels are raised in tuberculosis, infectious mononucleosis, viral hepatitis and malignancy. While values higher than 70U/l are highly sensitive and specific for pleural tuberculosis, values more than 100U/l highly suggest malignancy. Our patient had a pleural fluid ADA of 196.50 U/l and lead to suspect a malignancy.  In SLE the effusion is bilateral in 50% with a glucose of more than 50mg/dl, LDH of less than 500U/l, ANA>1:160. Pleural fluid ANA: Serum ANA >1 strongly suggestive of lupus pleuritic. Our patient had a pleural fluid LDH 1085U/l. Her ANA was negative. Familial Mediterranean Fever is an autosomal recessive disorder due to FMF gene mutation. It presents with recurrent attacks of fever and serositis. 95% of cases present with peritoneal inflammation rather than pleural inflammation as in our case. Variable involvement of pleura, pericardium, synovium and skin are reported. Acute phase reactants are elevated during the attacks. But generalized lymphadenopathy like in our case is uncommon.

Malignant etiology was proved in our case. Primary effusion lymphoma also called as body cavity lymphoma is seen primarily in HIV patients and associated with HHV-8 and EBV DNA. They don’t express surface markers for B or T cells and a thought to represent a preplasmacytic differentiation. Our patient had strong positivity for T cell lineage(CD 3) and was negative for B cell lineage(CD 20). Kikuchi-Fujimoto disease is a rare benign condition of unknown cause primarily affecting young women. It is characterized by fever, weight loss, SLE like rash and cervical lymphadenopathy. Usually it mimics malignancy but biopsy of lymph nodes show necrosis and follicular hyperplasia. It closely mimics Hodgkin lymphoma & SLE lymphadenitis. It is CD4 and CD8 positive. Our patient Is CD3 positive.

Hypothyroidism can cause pleural and pericardial effusion but lymphadenopathy and weight loss is contrary to its weight gain. Early suspicion of polyserositis and a thorough knowledge of its various etiologies can diagnose this rare syndrome earlier and can prevent death. Although we couldn’t save this patient, we gained knowledge and experience in handling such cases in future. This brought us forward to publish this case.

DISCLOSURE STATEMENT

None

REFERENCES

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