Age: 1.25
Sex: F
Vaccine: MMR
Symptoms: Laryngitis, Neutropenia, Pancytopenia, Pharyngitis, Pyrexia
Clinical narrative: Information has been received from a registered nurse and follow up information from physicians concerning a 15 month old white female patient with allergies to penicillin, amoxicillin (causes rash) and questionable allergy to azithromycin (may have caused rash) who was born at 36 weeks gestation and was on oxygen briefly immediately after birth, was hospitalized 8 days after birth for jaundice and had multiple ear infections during her first year of life. It was reported that there was no family history of rheumatoid arthritis, lupus, autoimmune disorder, immunodeficiency disorders or colitis in any family members. The patient was vaccinated on 12/15/95 with a first dose of MMR (second generation) (lot # not reported). Subsequently, in December 1995, the patient developed croup. On 12/26/95 the patient developed a very high fever and pancytopenia and was hospitalized. It was reported that all cell lines were down but the exact blood counts were unknown. In February 1996, the diagnosis of Evans Syndrome was made, which included autoimmune neutropenia, Coomb's positive hemolytic anemia and thrombocytopenia. During the course of her illness the patient had multiple admissions for neutropenia and fevers but it was reported that all cultures had all been negative. From diagnosis in February 1996 until November 1997, the patient required multiple medical therapies including steroids, danazol, vincristine and IVIG. A central line was placed for frequent blood draws and was removed in 1998. The patient had a good response to these treatments and has not required steroids, danazol, or vincristine since 1997. The patient's family reported that her platelet count had been running in the 180,000's from November 1997 until May 2000. In May 2000, the patient had a platelet count of 28, 000 that was associated with a severe stomach virus. This was felt to be an isolated ITP because her platelets were the only cell line affected. This resolved after a single dose of IV IgG. The patient's family stated the platelet count was in the 180,000's after this event. On 6/20/01 lab evaluation revealed a white count of 5.2, monocytes 8%, eosinophils 2%, hemoglobin 13.8, hematocri 40.4 and platelets 193,000. On 7/25/01 a physician reported that the patient stated she had been told that she had autoimmune hives (also reported as autoimmune urticaria). The patient has had a couple episodes of hives from head to toe with no clear etiology. The patient has been seen by a allergist who did extensive testing but did not find any specific allergens. It was also reported that the patient has had problems with frequent rashes on her inner thighs and the backs of her knees. The rash was pruritic, erythematous and a little bit scaly and seemed to be on the lower extremities only. The patient had also recently developed recurrent and persistent warts on the inner aspects of the knees and physical exam noted multiple flesh colored papules, 3-5mm in size over the medial aspect of her knees. The physician noted that the rashes and the warts could be a function of immune dysregulation and the physician questioned the possibility of a T-cell disorder. Lab evaluation on 7/25/01 revealed a white count of 5,800 mm3, hemoglobin of 13.5gm/dL, hematocrit of 38.5%, MCV of 78.3, platelet count of 199,000 mm3 and retic count of 2.3%. Peripheral blood smear showed normal white cell and platelet morphology, red cells showed no evidence of spherocytosis nor any other consistent abnormality. On 7/25/01 the patient still had mild reticulocytosis, which the physician noted could indicate some degree of ongoing hemolysis. Additional work up from 7/25/01 revealed ANA was negative, C3, C4, and CH50 were normal and IgG 447 (lower limit of normal noted to be 610). The physician noted that these results indicated that the patient may have a more generalized immunoregulatory abnormality, perhaps common variable immunodeficiency and the patient was referred to a pediatric immunology physician. Lab evaluation in August 2001 revealed IgG level of 481 mg/dL (normal 610/1230) and IgG2 <16 mg/dL (normal 30-380). Neither tetanus nor rubella antibody levels were in the protective range. in November 2001 the patient received a diphtheria and tetanus booster and lab evaluation in December revealed the anitbody levels for both diphtheria and tetanus were protected. On 2/26/02 a physician noted that although the patient's IgG level and IgG subclass 2 were low, they were encouraged by the patient's strong antibody response to immunizations. The physician noted they did not have any evidence that the patient's problem with Evans Syndrome was caused by the MMR vaccine and recommended that the patient receive vaccines for MMR and varicella. In February 2003 prior to receiving the varicella vaccine the patient developed varicella which manifested by prodromal fever and upper respiratory tract symptoms, followed by a pruritic rash. She was treated with acyclovir and she recovered within 2 weeks On 4/4/03 the patient was seen by a physician for routine follow up visit and it was noted that the patient had developed pharyngitis prior to the chicken pox and was treated with azithromycin. At this visit the physician noted that the patient was healthy and her IgG was now in normal range. The problems/diagnosis noted by the physician were remote history of Evans Syndrome and history of low IgG. Lab evaluation on 4/4/03 revealed WBC of 6640/cu mm, lymphocutes 2320, neutrophils 3790, hemoglobin 14 g/dL, platelets 201,000/cu mm. Immunoglobulin levels were normal with IgG of 612 mg/dL, IgA of 59 mg/dL and IgM of 170 mg/dL. Upon internal review, gastroenteritis was determined to be an other important medical event (OMIC). Follow up information received on 5/14/03 contained the following adverse experience: geographic tongue (onset unknown but noted on physical exam of 7/25/01). It was also reported that in August 2002 the patient had a negative titer following vaccination with Attenuvax. Additional information is not expected.