1031 This work was supported in part by FWGO-Belgium (contract 20.122). The help of Dr Sokal and Dr Michaux, Service d’Hematologie, Cliniques Universitaires St. Pierre, and of Dr Verwilghen and Dr Louwagie, Dienst Hematologie, A.Z. St. Rafael, Louvain, is gratefully acknowledged. Division of Human Genetics, Department of Human Biology, University of Leuven, Minderbroedersstraat 12, B-3000 Leuven, Belgium.
CONGENITAL CYTOMEGALOVIRUS INFECTION AND BILEDUCT OBSTRUCTION IN NEWBORN INFANT WITH CYSTIC FIBROSIS OF PANCREAS
SIR,-Infection with cytomegalovirus (c.M.v.) compli1% of pregnancies, and it has been detected as early as 20 weeks’ gestation.2 Although perinatal infection may be fatal or result in mental retardation, there are no detectable sequelx in the great majority of surviving infants. cates over
H.
VAN DEN
BERGHE.
MARKER-CHROMOSOME 14 IN MULTIPLE MYELOMA AND PLASMA-CELL LEUKÆMIA
SIR,-A consistent marker identified
as
a no.
14
(D-
chromosome with an extra terminal band has been found in 10 out of 12 Burkitt lymphomas.1 We wish to report a similar marker chromosome, a no. 14 with 2 extra bands terminally, identified with Giemsa banding, that occurred in two of our patients (see figure). Patient 1 His marrow had a modal had plasma-cell leukaemia. chromosome number of 46, and the abnormal no. 14 replaced a normal no. 14 in 6 out of 35 cells karyotyped. Other markers were also present, and the origin of the
group)
In the wake of the 1964 rubella epidemic there has been increasing awareness of congenital anomalies caused by chronic intrauterine infection.3 The following case-report illustrates several similarities. A Black male was born after 30 weeks’ gestation, with a birth-weight of 1300 g. At birth he had a petechial rash over his chest and shoulders, and developed hepatosplenomegaly and tachypnoea. No stools were passed and his abdomen became increasingly distended. Laboratory findings included a hasmatocrit of 38%, white blood-cells 3700 per c.mm., platelets 58,000 per c.mm., negative Coombs reaction, and bilirubin 16-5 mg. total, 5 mg. directreacting, per 100 ml. Inclusions suggesting C.M.v. infection were identified in cells in the urinary sediment. A diagnosis of meconium ileus was based on the demonstration of a microcolon on barium enema. On the 2nd day of life meconium ileus was confirmed at laparotomy and was managed by a gastrostomy and enterostomy. The infant’s
postoperative course was complicated by bradycardia, hypoxia, and acidosis. Despite corrective measures, he died on the 3rd postoperative day. Sweat was obtained prior to death, but the quantity was insufficient for determination of electrolyte concentrations. The relevant findings at necropsy included diagnostic lesions of cystic fibrosis in the pancreas, Brunner’s glands, and the genital tract. In addition, there was pronounced
hypersecretion
of
mucus
in the
mucosa
of the small and
large intestine and in the trachea and bronchi. Characteristic c.M.v. inclusions were seen in foci of the renal tubules, and more sparsely in single cells of the bronchial epithelium. Marker 14 from 4 cells of
(a) patient 1, (b) patient 2, and (c) control.
not be determined. Patient 2 had Her had a bimodal chromosome marrow multiple myeloma. count of 42 and 46. In all cells with 42 chromosomes (21 karyotyped) the abnormal no. 14 replaced a normal no. 14. There were other markers present, and the origin of the All cells with 46 extra bands could not be determined. chromosomes were normal. Both patients had free lambda light chains in the plasma and urine, and in each case the heat test for Bence-Jones protein was positive. A large acrocentric marker appearing in the absence of one normal D-group chromosome, and often referred to as the AM marker, was described in a number of cases of myeloma, plasmacytoma, and chronic myelogenous leukaemia before the advent of banding techniques in chromosome studies; it is unknown whether this marker represents a single entity. The significance of the findings reported here is not yet clear but suggests that banding may reveal that the no. 14 chromosome is affected in some hasmatological diseases more often than would be expected by chance
No evidence of necrosis was found at either site. The liver showed extensive extramedullary haematopoiesis, periportal aggregates of chronic inflammatory cells,
additional bands could
1. 2.
T. H. New Engl. J. Med. 1971, 285, 267. Rosenstein, D. L., Navarrete-Reyna, A. Am. J. Obstet. Gynec. 1964, 89, 220. 3. Esterly, J. R., Oppenheimer, E. H. Archs Path. 1969, 87, 380.
Weller,
alone. This work
was
supported by N.I.H.
Departments of Pathology, Maternal and Child Health.
Department of Medicine, Dartmouth Medical School, Hanover, New Hampshire 03755 U.S.A. 1.
grant HD 03298.
D. H. WURSTER-HILL. O. R. MCINTYRE G. G. CORNWELL, III L. H. MAURER.
Manolov, G., Manolova, Y. Nature, 1972, 237, 33.
Fig. 1-Portal triad showing several bile ductules obstructed by enlarged hyperplastic epithelium containing intranuclear inclusions of C.M.V. infection.
(Haematoxylin and
eosin. Reduced
by a quarter from
x
410.)