Tuberculosis associated haemophagocytic syndrome.

HS Subhash, S Sowmya, U Sitaram, AM Cherian 
 

Correspondence Address:
H S Subhash

Full Text

Sir,

A twenty-nine year old lady presented with six-month history of intermittent fever, chills, anorexia and weight loss. She also had history of diffuse abdominal pain and progressive abdominal distension of two months duration. Her past medical and family history was unremarkable.

On physical examination there was significant pallor. Respiratory system examination showed moderate right-sided pleural effusion. Per abdominal examination showed moderate ascites and an enlarged liver (3-cm below the costal margin). Other systemic examination was normal.

Investigations showed Hb 6.9 gm%, total white blood cell count of 3900 cu/mm, differential count neutrophils 78%, lymphocytes 14%, eosinophils 1%, monocytes 1%, band forms 6%. reticulocytic count 1.9%, ESR 70 mm at 1 hour, platelets 4,37,000/cumm. Liver function tests, serum electrolytes, creatinine, random plasma glucose and uric acid were normal. HIV, HCV, HBsAg, dengue serology, smear for malarial parasites, rheumatoid factor and ANA were negative. Urine microscopy and stool examination was normal. Ultrasonogram of the abdomen showed multiple mesenteric, peripancreatic, paraaortic, portocaval lymphnodes, moderate ascites, right sided pleural effusion and enlarged liver. Chest x-ray showed right-sided pleural effusion. ECG, Echocardiogram and gastroscopy were normal. Ascitic fluid and pleural fluid examination showed exudative type of effusion. Cytology examination of the ascitic and pleural fluid and lymphoma markers of the pleural fluid was negative. Pleural fluid, ascitic fluid and bone marrow smear for AFB was negative. Pleural biopsy showed caseating granuloma consistent with tuberculosis.

Bone marrow examination showed hypercellular marrow with erythrophagocytosis. She was started on anti-tuberculous medications following that she showed a remarkable improvement during follow-up visits.

Haemophagocytic syndrome (HPS) is a disorder characterised by a benign proliferation of mature histiocytes along with uncontrolled phagocytosis of some haematic precursors in the bone marrow.[1] It is best known to be associated with viral infection but other associated diseases have also been implicated including tuberculosis (TB).[2],[3],[4],[5]

HPS has been reported in several clinical situations and has been classified into malignant and benign reactive type. The main etiological agents involved in the reactive HPS are several viruses like herpes simplex, varicella-zoster, Epstein-Barr, cytomegalovirus, adenovirus, parainfluenza and rubella. Non-viral causes include leishmaniasis, Q fever, brucellosis, fungi, gram-positive and gram-negative bacteria, HIV infection and drugs have also been reported.[2]

Among patients presenting with features of HPS, published reports suggest that in some cases tuberculosis is diagnosed only after postmortem examination.[2],[5] Virus associated HPS, though potentially a reversible condition is noted to have a high mortality of about 30 to 40%.[5] Tuberculosis associated HPS carries a higher mortality than virus related HPS. It is noted that survival rate is only 16.7% among HIV infected patients along with tuberculosis related HPS.[1] This case is presented to highlight that TB should be considered in patients with HPS specially in our country because of higher prevalence of this disease.

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