A 23 year old male patient presented with complaints of swelling of both lower limbs and abdominal distension of one week duration. He was not an alcoholic. There was no history of neuro-psychiatric symptoms or any other significant complaints. Patient had history of jaundice at the age of two.
Examination revealed bilateral pitting pedal edema. There was no icterus. Incidentally, he was found to have bilateral cervical, axillary, inguinal lymph nodes of approximately 1-1.5 cm each, which were non-tender. There were no Kayser-Fleischer rings on slit lamp examination. Patient had firm, nontender hepatomegaly, moderate splenomegaly and moderate ascites. Other systems were clinically normal.
With this constellation of symptoms and signs, a differential diagnosis of a haematological malignancy (leukemia or lymphoma) or disseminated tuberculosis was considered.
Investigations - Hb 13.3 g/dl, Total WBC Count 19,400 cells/cumm, Differential of N78L16E04, ESR 25 mm/hr, Platelet count 2.11 lakhs /cumm. Urine had NO albumin or sugar and 1-2 pus cells and 1-2 epithelial cells.
RBS 104 mg/dl, Blood urea 24 mg/dl, Serum creatinine 1.0 mg/dl. Liver function tests revealed Total bilirubin 1.0 mg/dl , Direct bilirubin 0.70 mg/dl, Total protein 6.0 g/dl, Serum Albumin 3.3 g/dl , A:G ratio 1.1, AST 45, ALT 33, Alkaline phosphatase 419. Prothrombin time INR was 1.25, APTT 1.29. Bleeding time 3’00” and Clotting time of 4’00”.
Ascitic fluid was analysis revealed a cell count of 50 cells/cumm, Cell type predominantly lymphocytes, few neutrophils and occasional mesothelial cells. Gram’s stain showed few lymphocytes but no organisms. AFB stain was negative for acid-fast bacilli. Ascitic fluid Glucose was 126 mg/dl, protein 0.8 g/dl. SA-AG (Serum Albumin-Ascitic albumin) gradient was 2.5, suggestive of transudative ascites.
Viral markers (HBsAg, Anti HCV) were negative. HIV was negative. Anti Nuclear antibody test was negative. Serum Ceruloplasmin 23.4 mg/dl α1-Antitrypsin 2.56g/l. Ultrasound abdomen showed slightly enlarged liver with heterogenous echotexture, multiple ill-defined hypoechoic mass lesions in both lobes, mild splenomegaly, Moderate ascites. Features suggestive of Chronic liver disease to rule out underlying malignancy.Upper GI endoscopy showed Grade 1 Esophageal varices and Portal Hypertension. CT scan abdomen showed Nodular surface of liver, mild to moderate ascites, Multiple enlarged mesenteric lymph nodes, mildly enlarged spleen. Other abdominal organs were normal. CT features were suspicious of cirrhosis of liver with ascites and with mesenteric lymphadenopathy.
FNAC of cervical lymph node showed lymphoid series of cells in various stages of maturation; many histiocytes, occasional giant cells and plenty of eosinophils. Liver biopsy showed ballooning degeneration of hepatocytes with loss of normal architecture and formation of pseudolobules. Fibrous septae with foci of cholestasis was also seen. Reticulin staining showed increase in fibroblasts. All these features were suggestive of Cirrhosis of liver.
In view of inconclusive FNAC report, Axillary lymph node excision biopsy was undertaken. Histopathological study of which revealed marked hyperplasia of follicles, large number of eosinophils, neutrophils and plasma cells. Many hyalinised blood vessels were seen- features suggestive of Kimura disease.
Hence a final diagnosis of Cirrhosis liver probably cryptogenic post infective etiology with Kimura disease was arrived at. The patient was put on a low-salt fluid restricted diet along with diuretics with which he symptomatically improved. Kimura disease warranted no treatment in him at this stage.
Kimura disease
Kimura disease is a rare benign chronic inflammatory disorder. It was first described in 1937 (Kim et al) from China and later popularized in 1948 by Kimura and his associates who noted a change in the surrounding blood vessels and referred to it as "unusual granulation combined with hyperplastic changes in lymphoid tissue."
Kimura Disease is predominantly seen in males of Asian descent. The cause of Kimura disease remains unknown. Reasons like an allergic reaction or an alteration of immune regulation are suspected. Other theories like persistent antigenic stimulation following arthropod bites and parasitic or candidal infection have also been proposed. To date, none of these theories have been substantiated.
It typically presents as non-tender subcutaneous swelling in head and neck region, predominantly in preauricular and submandibular area. It is associated with lymphadenopathy both local and distal, marked peripheral eosinophilia and an elevated IgE level. The disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium.
Renal involvement is the only systemic manifestation which may affect up to 60% of patients as membranous glomerulonerphritis, minimal change glomerulonephritis, diffuse proliferative glomerulonephritis, mesangial proliferative glomerulonephritis and also nephrotic syndrome (12% of cases).
Differential diagnosis include
.
· Eosinophilic granuloma
· Mikulicz's disease
· Acute lymphocytic leukaemia
· Hodgkins disease
· Angioimmunoblastic lymphadenopathy
It can easily be mistaken for a malignant disorder. FNAC is misleading and diagnosis is established only on histopathological examination.
Treatment: Observation is acceptable if the lesions are neither symptomatic nor disfiguring. Intralesional or oral steroids can shrink the nodules but seldom result in cure. Cetirizine is an effective agent in treating the symptoms of Kimura's disease. Radiotherapy has been used to treat recurrent or persistent lesions. However, considering the benign nature of this disease, radiation should be considered only in cases of recurrent, disfiguring lesions. Surgery has been considered the mainstay of therapy. However, recurrence after surgery is common.
Kimuras Disease should be considered in differential diagnosis in patients who present with primary lymphadenopathy with Eosinophilia and investigated accordingly as the disease has an indolent course and good prognosis.
Acknowledgements
We sincerely thank Dr.Manjunath, Professor & Head Pathology, Dr.Sunila, Professor of Pathology for their help extended during work-up of this case.
References
1. Kim HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz’s disease.Chin Med J 1937; 23:699-700.
2. Kimura T, Voshimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissues. Trans Soc Pathol Jpn 1948; 37:170-80.
3. Messina T. M. D., Armstrong W. B., Pena F. et al (1998) : Kimura's disease two case reports and a literature review. Annals of Otology, Rhinology and Laryngology 107 (12) : 1066-71.
4. Kung I. T. M., Gibson J. B., Bannatyne P. M. (1984) : Kimura's disease : A clinico-Pathological study of 21 cases and its distinction from angiolymphoid hyperplasia and eosinophilia.Pathology 16 : 39-44.
5. Googe PB, Harris NL, Mihm MC Jr. Kimura’s disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. J Cut Pathol 1987; 12:263-71.
6. Chusid M. J., Rock A. L., Sty J. R. et al (1997) : Kinmura's disease : an unusual cause of cervical tumor. Archives of disease in childhood 77 (2) : 153-4.
No comments:
Post a Comment