VENEREAL DISEASE

Anemia as the Only Presenting Manifestation of Congenital Syphilis André D. Lascari, M.D., Jess Diamond, M.D., Brian E. Nolan, M.D.

ANEMIA

f~i~I~EMIA is

a common

but

manifestation of is the

rarely congenital syphilis, only presenting feature of the disease. A threemonth-old infant is reported who had anemia of sudden onset with no other feature suggestive of congenital syphilis. Case Report A three-month-old white male was admitted to St. John’s Hospital, Springfield, for evaluation of anemia. The infant had been seen by one of us (J.D.) six days previously because of a cough; hemoglobin and hematocrit at that time were I I gm/100 ml and 31 per cent, respectively. The mother was in another hospital at this time for a vaginal hysterectomy and not available for a history. The grandmother stated that for the past four days the infant had become pale and the stools had been black. He cried less than usual and his hands were usually cold. An initial diagnosis of gastrointestinal bleeding was made. The infant was born to a gravida 9, para 5, Ab 4, 30-year-old mother. The birth weight was 4.0 kg. The infant had an imperforate anus; an anoproctoplasty was performed and the infant was discharged in good condition. The blood type of the mother and infant was A Rh positive. The Coombs test on the baby’s blood was negative. A barium swallow and upper gastrointestinal contrast study were normal. The mother’s rapid

test (R.P.R.) for syphilis was nonreactive at the time of birth. . At three months of age, the infant was very pale, but in no distress; he weighed 4.9 kg. The conjunctivae and mucous membranes were very pale.

plasma reagin

Department of Pediatrics, Southern Illinois University School of Medicine, P.O. Box 3926, Springfield, Ill. 62708.

The tip of the spleen could be palpated, but its size was deemed within normal limits for age. The liver was not enlarged. There was no rhinitis, rash, lymphadenopathy, or limitation of movement of the extremities. The blood hemoglobin level was 7.6 gm/100 ml; the smear showed normochromic, normocytic red cells and abundant platelets. The hematocrit was 2 L7 per cent; reticulocyte counts 2.? per cent; WBC 8,400 with 47 per cent PMI’~s, 46 per cent lymphocytes, 5 per cent monocytes, and 2 per cent eosinophils. The results of the following studies were normal: chest roentgenogram, partial thromboplastin time, prothrombin time, T-3, T-4, blood urea nitrogen, total serum protein, serum albumin, serum glutamic oxaloacetic transaminase, calcium, phosphorus, bilirubin, direct Coombs test, and urinalysis. Stools were negative for occult blood on five successive days. Serum iron concentration was 15 >g/100 ml (normal 65-175 ~g/100 ml), total iron binding capacity was 278 ~g/100 ml (normal 250-410 ,u.g/100 ml) and the per cent saturation was 5.4 (normal 20-5~%}. The serum haptoglobin value was 250 mg/100 ml (normal 105-153

mg/100 ml).

The initial impression of gastrointestinal bleeding was not confirmed and the infant was thought to have a hemolytic anemia. The hemoglobin level continued to drop; it was 6.5 gm on the fifth hospital day, and the reticulocytes were elevated to 5.4 per cent. At this time, the mother’s physicians called and stated that her serology, which had been negative at birth, was now positive (R.P.R. reactive 64 dil., fluorescent treponemal antibody

were then taken of the infant’s extremities; the classical Wimberger’s sign was present (focal erosion of the medial aspects of the proximal tibias). The R.P.R. of the infant was reactive at 64 dils. and the F.T.A. was reactive. There were l.l red blood cells and 8 segmented cells in the spinal fluid and

(F.T.A.) -reactive). Roentgenograms

90

Downloaded from cpj.sagepub.com at The University of Iowa Libraries on June 3, 2015

the protein concentration was 59 mg/100 ml; the Venereal Disease Research Laboratory test (VDRL) on the spinal fluid was reactive and the colloidal gold curve was 0122210000. The infant was transfused with 75 ml of packed red cells when the diagnosis of congenital syphilis was confirmed. He was then given 200,000 units

procaine aqueous penicillin intramuscularly, daily for ten days. His course was afebrile except for a spike in temperature to 38.9 C four hours after the first injection of penicillin. Three months after diagnosis and transfusion the hemoglobin concentration was 13.5 gm/100 ml and a screening test (DADE) for glucose-6-phosphate dehydrogenase

(G6PD)

was

puncture is planned initial

course

normal. A repeat lumbar at six months after the

of penicillin.

Discussion This case was puzzling to all the physicians involved because the diagnosis of congenital syphilis was never considered. It was only the fortuitous hospitalization of the mother for surgery and the serologic study which led to the diagnosis in the infant. The infant had none of the typical physical findings of the disease: no snuffles, pseudo-

paralysis, hepatosplenomegaly, lymphadenopathy, pneumonia, diaper rash, mucolesions about the mouth, anus, or genitalia, maculopapular rash. 1.2 No reof instances of congenital syphilis similar ports presenting only with anemia and no other physical sign of the disease could be found in the literature. The anemia of congenital syphilis has been attributed variously to hemolysis, interference with hematopoiesis, hypersplenism, and nutritional deficiency.3 -’ Hemolysis was adjudged the primary cause of anemia in eight of nine infants with congenital syphilis in one reported serieS.3 Documentation of a sudden drop in hemoglobin concentration of 4.5 gm in eleven days, in the absence of demonstrable blood loss, strongly suggested a hemolytic process in our patient. An elevated serum haptoglobin does not rule out hemolysis, since its level may be normal or increased in the presence of hemolysis and an inflammatory process.~ The low serum iron concentration combined with a low total iron binding capacity, also most likely reflects the inflammatory proceSS.6 An elevated indirect cutaneous

or

bilirubin level was not present but this value is often normal with mild degrees of hemolysis; some elevation would be expected in our infant, however, if the sudden decrease in hemoglobin was due to a hemolytic process. This case also illustrates the point that a negative serology test for syphilis in the mother or infant at the time of birth does not invariably rule out congenital syphilis. 7.8 When the infection is acquired late in gestation, the specific tests may not become positive in the mother and infant until after delivery. The delayed onset of the initial symptom in our infant also suggests that the infection was acquired -Iate in gestation.9 A number of recent articles have stressed the alarming increase in congenital syphilis and the failure of many physicians to consider the diagnosis in infants with findings suggestive of the disease.2.8.10.11 We would advise that a serologic test for syphilis be performed with every sick infant, whether or not the typical findings of congenital syphilis are present. Congenital syphilis should also be routinely included in the differential diagnosis of anemia in infancy. serum

References 1.

Nelson, Waldo E.,

Victor C., III and Eds.: Textbook of Pediatrics, 9th ed. Philadelphia, W.B. Saunders, 1969.

McKay, 2.

3.

R.

Vaughan,

James,

Woody, Norman C., Sistrunk, William F., and Platou, Ralph V.: Congenital syphilis: a laid ghost walks.

J. Pediatr. 64: 63, 1964. Whitaker, Jo Anne, Sartain, Peggy, and Shaheedy, Mohamad:

Hematological aspects of congenital Pediatr. 66: 629, 1965. Sartain, Peggy: The anemia of congenital syphilis. Southern Med. J. 58: 27, 1965. Owen, J. A., Smith, R., Padanyi, R., and Martin, J.: Serum haptoglobin in disease. Clin. Sci. 26: 1, 1964. Cartwright, G. E.: The anemia of chronic disorders. Semin. Hematol. 3: 351, 1966. Curtis, Arthur C., and Philpott, Osgoode S., Jr.: Prenatal syphilis. Med. Clin. North Am. 48: 707, 1964. Saxoni, Fotini, Lapatsanis, Peter, and Pantelakis, Stephanos N.: Congenital syphilis: a description of 18 cases and re-examination of an old but ever-present disease. Clin. Pediatr. 6: 687, 1967. Sparling, P. Frederick: Diagnosis and treatment of syphilis. N. Engl. J. Med. 284: 642, 1971. Wilkinson, Robert H., and Heller, Richard M.: Congenital syphilis: resurgence of an old problem. Pediatrics 47: 27, 1971. Hoffmann, F. Donald, and Herweg, John C.: Status of serological testing for congenital syphilis. J. Pediatr. 71: 686, 1967.

syphilis. J.

4.

5. 6.

7.

8.

9. 10.

11.

91

Downloaded from cpj.sagepub.com at The University of Iowa Libraries on June 3, 2015

Anemia as the only presenting manifestation of congenital syphilis.

VENEREAL DISEASE Anemia as the Only Presenting Manifestation of Congenital Syphilis André D. Lascari, M.D., Jess Diamond, M.D., Brian E. Nolan...
227KB Sizes 0 Downloads 0 Views