Case 19-2003: a Five-Day-Old Girl with Leukocytosis and a Worsening Rash from Birth
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The new england journal of medicine case records of the massachusetts general hospital Founded by Richard C. Cabot Nancy Lee Harris, m.d., Editor Sally H. Ebeling, Assistant Editor William F. McNeely, m.d., Associate Editor Stacey M. Ellender, Assistant Editor Jo-Anne O. Shepard, m.d., Associate Editor Christine C. Peters, Assistant Editor Case 19-2003: A Five-Day-Old Girl with Leukocytosis and a Worsening Rash from Birth Lawrence C. Wolfe, M.D., Howard J. Weinstein, M.D., and Judith A. Ferry, M.D. presentation of case A five-day-old girl was admitted to the hospital because of a worsening rash from birth From the Division of Pediatric Hematolo- and leukocytosis with blasts. gy/Oncology, Floating Children’s Cancer Center at Tufts New England Medical Cen- The infant was delivered at 40 weeks’ gestation by a 24-year-old woman (gravida 2, ter, and the Department of Pediatrics, Tufts para 2), who had immigrated from Central America to the United States two months ear- University School of Medicine (L.C.W.); lier. The pregnancy had been complicated by a urinary tract infection and by a vaginal the Division of Pediatric Hematology and Oncology, Massachusetts General Hospi- discharge, which had been treated with topical medication. tal for Children (H.J.W.); the Department The mother had no history of oral or genital herpes, sexually transmitted diseases, or of Pathology, Massachusetts General Hos- varicella during the pregnancy. Ultrasonographic examinations of the fetus, reportedly pital (J.A.F.); and the Departments of Pe- diatrics (H.J.W.) and Pathology (J.A.F.), performed at four and seven months’ gestation, revealed no abnormalities. Delivery was Harvard Medical School — all in Boston. spontaneous and without complications; the newborn infant weighed 3.6 kg. At birth, an erythematous facial rash was present and was suspected to be erythema toxicum ne- N Engl J Med 2003;348:2557-66. onatorum. On the second day of life, the infant was discharged. The rash progressively Copyright © 2003 Massachusetts Medical Society. worsened, and marked, bilateral periorbital swelling and erythema developed. There was a yellow discharge from the lesions, and the rash spread to the abdomen and diaper area. On the fifth day of life, the infant was referred to another hospital for evaluation. There was no fever, and she was active, alert, and feeding normally. The urine was nor- mal. Hematologic and other laboratory data are shown in Table 1. A diagnosis of perior- bital cellulitis was made, and after the administration of ampicillin and gentamicin, the infant was transferred to this hospital. The patient’s mother had a history of a positive tuberculin skin test (induration, 20 mm in diameter), but a chest radiograph had been clear, and no prophylaxis had been given. Prenatal screening tests for antibodies to the human immunodeficiency virus (HIV) and hepatitis B antigen were negative; a prenatal serologic test for syphilis was also negative. The mother was immune to rubella. There was no information about the results of a vaginal culture for group B streptococci. The patient had a sister, eight years old, who resided with her mother in the United States; her father remained in Central America, and no other family members were in the United States. There was no maternal history of consanguinity, birth defects, recurrent miscarriage, stillbirth, or mental retar- dation. The mother had no information about the father’s family history. On admission, the temperature was 37.2°C, the pulse was 144 beats per minute, and the blood pressure was 105/80 mm Hg. The weight was 3.8 kg (50th to 75th percen- tile). The oxygen saturation was 99 percent. n engl j med 348;25 www.nejm.org june 19, 2003 2557 The New England Journal of Medicine Downloaded from nejm.org at The University Of Illinois on February 1, 2017. For personal use only. No other uses without permission. Copyright © 2003 Massachusetts Medical Society. All rights reserved. The new england journal of medicine On physical examination, the infant was slen- Table 1. Laboratory Data.* der; she appeared to be comfortable and well, with- Referring On 2nd out dysmorphic features. Erythematous and vesic- Variable Hospital Admission Hospital Day ulopustular lesions were present around the eyes, cheeks, and chin, and there was periorbital edema Hematocrit (%) 41.1 44.8 40.5 3 (Fig. 1). Some of the lesions were crusted. Vesicu- White cells (per mm ) 64,000 73,200 74,100 lopustular lesions were also evident on the chest Differential count (%) and thighs, and there were pustular lesions in the Neutrophils 12 13 16 inguinal area and on the labia. The mucous mem- Lymphocytes 25 19 24 branes were normal. No lymphadenopathy was found. The lungs and heart were normal. The liver Atypical lymphocytes 2 1 edge descended 5 cm below the right costal margin, Monocytes 2 1 and the splenic tip was palpable. On neurologic ex- Band forms 5 3 amination, the primitive reflexes were intact, and Eosinophils 1 1 no focal deficits were detected. Blasts 62 52 54 The levels of urea nitrogen, uric acid, calcium, Myelocytes 4 1 Metamyelocytes 2 Platelets (per mm3) 700,000 973,000 949,000 A Reticulocytes (%) 3.5 Prothrombin time Normal Partial-thromboplastin time Normal Mean corpuscular volume (µm3) 109 Cerebrospinal fluid Clear, colorless Red cells (per mm3) 140 White cells (per mm3) 10 Mononuclear cells (%) 76 Lymphocytes (%) 12 Blasts (%) 11 Neutrophils (%) 1 Glucose (mg/dl) 57 56 78 B Total bilirubin (mg/dl) 2.3 Conjugated bilirubin (mg/dl) 0.8 Protein (g/dl) 57 Normal Albumin Normal Globulin Normal Sodium (mmol/liter) 138 Potassium (mmol/liter) 6.9 Chloride (mmol/liter) 103 Urea nitrogen (mg/dl) Normal 7 Creatinine (mg/dl) 3 0.2 Lactate dehydrogenase (U/liter) 1,992 * To convert the values for glucose to millimoles per liter, multiply by 0.05551. Figure 1. Photographs of the Patient on Admission. To convert the values for total and conjugated bilirubin to micromoles per liter, There is a prominent, vesiculopustular rash on the face multiply by 17.1. To convert the value for urea nitrogen to millimoles per liter, mul- and periorbital swelling (Panel A). Vesicles and pustules tiply by 0.357. To convert the values for creatinine to micromoles per liter, mul- are also present on the torso (Panel B). tiply by 88.4. 2558 n engl j med 348;25 www.nejm.org june 19, 2003 The New England Journal of Medicine Downloaded from nejm.org at The University Of Illinois on February 1, 2017. For personal use only. No other uses without permission. Copyright © 2003 Massachusetts Medical Society. All rights reserved. case records of the massachusetts general hospital phosphorus, total protein, albumin, globulin, elec- found lesions that were consistent with herpes sim- trolytes, aspartate aminotransferase, alanine amino- plex, with little or no conjunctivitis and no chorio- transferase, and alkaline phosphatase were normal. retinitis. Additional laboratory data are shown in Table 1. The A diagnostic procedure was performed. peripheral-blood smear is shown in Figure 2. On a chest radiograph that did not include the differential diagnosis apexes of the lungs, the lung fields were clear, and the heart size was normal; there was no evidence of Dr. Lawrence C. Wolfe: The greatest challenge in this soft-tissue fullness in the left upper abdominal case is to establish a single diagnosis that reconciles quadrant, which would suggest splenomegaly. the seemingly paradoxical aspects of the patient’s A dermatologic consultant performed a Tzanck test, presentation. Multiple dire processes were possible, which was inconclusive. Preliminary results of a di- including life-threatening infection from a viral or rect fluorescence antibody test for varicella–zoster bacterial source with a leukemoid reaction in the virus and herpes simplex virus types 1 and 2 were peripheral blood, a congenital leukemia, or both, negative, and the results of cultures for varicella– occurring simultaneously. There was cerebrospinal zoster virus and herpes simplex virus were pending. fluid pleocytosis, which could indicate sepsis in a Specimens of blood and urine and swabs from child who otherwise appeared to be well or congen- ocular lesions were obtained for culture, and a spec- ital leukemia in a child with a platelet count as high imen of cerebrospinal fluid was sent for polymer- as 900,000 per cubic millimeter. Finally, the infant ase-chain-reaction analysis for herpes simplex virus. had a rash that started initially as the innocent- Fluids containing glucose and sodium bicarbon- appearing erythema toxicum but that blossomed ate were administered intravenously, as were vanco- into an intense vesiculopustular dermatitis that pro- mycin, cefotaxime, gentamicin, and acyclovir; vidar- gressed to periorbital cellulitis. abine ophthalmic ointment was applied to the eyes, Table 2 presents the salient features of the case. and allopurinol was given orally. Breast-feedings Table 3 applies them to a differential diagnosis that were begun. The temperature was normal on all revolves primarily around the discrimination of a occasions. leukemoid reaction due to infection and congenital The results of laboratory tests performed on the leukemia. The case history allows us to rule out sev- second hospital day are shown in Table 1. No addi- eral of these possibilities. The mother had a posi- tional laboratory findings were available from the tive tuberculin skin test, but she had no evidence referring hospital. An ophthalmologic consultant of pulmonary disease, dramatically decreasing the likelihood of perinatal transmission of tuberculo- sis. Neonatal syphilis and rubella must be ruled out in any consideration of leukemia in an infant; the mother’s negative serologic test for syphilis and her Table 2. Important Features on Admission. Progressive vesiculopustular rash, predominantly affect- ing the face and spreading High white-cell count, with a large percentage of blasts Elevated platelet count Cerebrospinal fluid pleocytosis with blasts Markedly elevated lactate dehydrogenase level (1992 U/liter) Figure 2.