Diagnostic Dilemma

Is it NK cell deficiency_?

A 3½ years old girl born of third degree consanguineous marriage presented in Dec 2014 with recurrent fever for the past 8 months. She had suffered from liver abscess in March 2014 {ultrasound showed multiple micro abscesses in both lobes of liver}. In August 2014, she had jaundice with ascites and right sided pleural effusion. Her HIV, HBsAg, Hepatitis C Elisa were negative and ultrasound {USG} abdomen showed hepatosplenomegaly with ascites and lymph node at porta. Doppler of portal system was normal. In September 2014, she had right sided otitis media with ear discharge. On presentation to us, her weight was 14.6 kg. She had insignificant cervical lymph nodes and hepatosplenomegaly. Other systems were normal. Investigation revealed normal nitroblue tetrazolium {NBT} test {98 percent}, serum ACE levels of 56.8 U, L {Normal = 8 to 52 U, L} and negative Quantiferon TB Gold test. Her lymphocyte subset analysis revealed low NK cells {CD16, 56 = 79 {2 percent} cells, cumm {Normal = 130-720 cells, cumm}, CD19 = 904 cells, cumm, CD4 = 1690 cells, cumm, CD8 = 943 cells, cumm and CD3 of 2948 cells, cumm} suggestive of NK cell deficiency. Her liver function tests were normal. She was started on Cotrimoxazole and advised regular follow up.

Is this NK cell deficiency_?
Expert Opinion :
Natural killer {NK} cells are lymphocytes of the innate immune system. They predominantly act against viral infections and in tumor cell surveillance. The viruses that seem to be best targeted by NK cell mediated defenses are those of the Herpesvirus family. There are 2 major types of NK cell deficiencies {NKD}. Classical NKD {CNKD} is defined as an absence of NK cells and their function among peripheral blood lymphocytes. Functional NKD {FNKD} is defined as the presence of NK cells within peripheral blood lymphocytes, having defective NK cell activity. To diagnose CKND, NK cells should be =1 percent of peripheral blood lymphocytes. Most patients present with varicella, cytomegalovirus {CMV}, Epstein Barr virus {EBV} and herpes simplex virus {HSV} infections. This child has not had such infections. Thus it is unlikely to be NKD. Her repeat lymphocyte subset after 3 months was normal.
Answer Discussion :
she is a case of combined immunodefficiency
7 years ago
Ahmed Fadel
no it look as chronic disseminated infection started in liver
7 years ago

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