Sex: M
Vaccine: HIBV
Symptoms: Blood immunoglobulin A normal, Blood immunoglobulin G normal, Blood immunoglobulin M decreased, Influenza virus test negative, Neutrophil count abnormal
Clinical narrative: poor response to Haemophilus influenzae type b; This literature marketed report has been received from the authors of a published literature article, titled as stated below and refers to a 14-year-old male patient, referred for lymphopenia, recurrent herpes stomatitis, and recurrent otitis media. He had a vesicular eruption with fever after varicella vaccination and developed shingles at age 3. He had chronic rhinitis and cough with negative testing to environmental allergens. At age seven he had serum immunoglobulin G (IgG) at 1400 mg/dL, serum immunoglobulin M (IgM) at 19.5 mg/dL and serum immunoglobulin A (IgA) at 43.1 mg/dL. The patient also had marked diminution in all lymphocyte subsets as provided below. At the age of 4, the patient had the following test results: CD3+ lymphocyte T cells: 193 per milliliter of blood, CD3+ lymphocyte T cells/ CD4+ subset: 84 per milliliter of blood, CD3+ lymphocyte T cells/ CD8+ subset: 113 per milliliter of blood, CD19+ B cells: 80 per milliliter of blood, CD3- CD16+ CD56+ Natural killer (NK) cells 87 per milliliter of blood, antigen proliferation (Candida) : 1.3, SI was low, however, basal proliferation was high, skewing interpretation of results. At the age of 10, the patient's mitogen proliferation - phytohemagglutinin (PHA) test was normal.  On an unknown date, the patient was vaccinated with Haemophilus b Vaccine (manufacturer unknown) (dose, route of administration, anatomical location, lot number and expiration date were not provided). At the age of 14, the patient had the following tests results: CD3+ lymphocyte T cells: 137 per milliliter of blood, CD3+ lymphocyte T cells/ CD4+ subset: 47 per milliliter of blood, CD3+ lymphocyte T cells/ CD8+ subset: 90 per milliliter of blood, CD19+ B cells: 33 per milliliter of blood, CD3- CD16+ CD56+ Natural killer (NK) cells 75 per milliliter of blood. Abnormal neutrophils were apparent by light microscopy. Large vacuoles were present in many of the neutrophils as well as some lymphocytes. These vacuoles were clearly seen by transmission electron microscopy. The patient had also "toxic vacuolation" on a manual complete blood count. His cells showed enlarged macropinocytotic vesicles in lymphocytes as well as neutrophils. On an unknown date (also reported as "at age 14"), the patient had normal to elevated IgG (1110 mg/dL), low IgM (11.9 mg/dL), and normal IgA (53.6 mg/dL), with marked diminution in all lymphocyte subsets.  On an unknown date, antibodies to tetanus, varicella, and a few pneumococcal serotypes were adequate with poor response to Haemophilus influenza type b. Targeted in-house immunodeficiency gene panel at Hospital identified de novo heterozygous RAC2 c.184G.A, p.E62K. The outcome of the decreased therapeutic response to Haemophilus influenza type b was not provided.  The reporter considered the event to be related to Haemophilus b vaccine (manufacturer unknown).    This is one of two reports derived from the same literature article and concerning the same patient cross-referenced with case MARRS# 1907USA003039).; Sender's Comments:  US-009507513-1907USA003039: