Sickle cell anemia with pregnancy

SICKLE CEI;L .ANEMIA WITH PREGNANCY ALBERT

\V.

LBWJS,

JR,., M.D., ST. AUGUSTINE, FLA.

(From the Departments of ]{edicine and of Obstetrics, Enwry Uni,versity School of Medicine and Grady Hospital, Atlanta, Georgia) cell anemia has :figured largely in medical literature since Herrickl deSICKLE scribed the condition in and there hav-e been many cases reported. kll.nder-

1910, son and vVare,2• 3 four years ago, published an excellent summary of the disease, presenting the cases observ-ed at Grady Hospital up to that time. Sickle cell anemia with pregnancy is rare. Lash4 reported such a case in 1934, :finding only two earlier ones in the literature. The following case seems to be the fourth. REPORT OF CASE

A negro woman, aged twenty-fiv-e years, was admitted to the Obstetric Service of the Emory University Div-ision of Grady Hospital on Nov. 13, 1935. Her last menstrual period had begun on Aug. 13, 1935. History.-As a child the patient had been unable to carry on normal activity beeause of fatigue and shortness of bTeath on moderate exertion. Heart disease had :first been diagnosed when she was ten. Two years later pain in both ankles had been severe enough to keep her in bed a week, although there was no swelling or fever. With limited aetivity she then became free of all symptoms until her marriage in 1931. During the next four years three pregnaneies had terminated in abortions at four to seven the last of these occurring during an illness, said to have been pneumonia. On Aug. 26, 1935, she was admitted to the medical outpatient department. A diagnosis of rheumatic heart disease was made and restricl;ion of activity was advised. 'fhe patient returned on Oct. 19, 1935, complaining of shortness of breath and palpitation. Hemoglobin at this time was 40 per cent (Tallqvist); red cells numbered 1,590,000; white cells 20,000. MaTked anisocytosis and poikilocytosis were observed. She was referred to the antepartum clinic because of amenorrhea for two months. There was no history of leg ulcers or abdominal pain. The family history was appaTently irrelevant.

Examination.-The patient was well dev-eloped but undernourished. Her face ap· peared somewhat puffy. The conjunctiva and mueous membranes .were extremely pale. Ophthalmoscopic examination was negative. Breast secretion was present. The left border of cardiae dullness was 11 em. from the midsternal line in the fifth intercostal space. The right border of dullness was 4 em. out in the fourth. No thrill was present. There was a diastolic shock at the base. The :first and second sounds at the apex were deaT and of good quality. There was a soft, blowing, systolic murmur at the apex transmitted to the base. The pulmonic second sound was aceentuated. The radial pulses were equal, synchronous, and of good v-olume. The fundus uteri was palpable 5 em. above the symphysis pubis. The liver and spleen were not felt. The cervix was softened. Goodell's and Hegar 's signs were present. The uterus was symmetrically enlarged and softened. The extremities presented no scars or ulcers. Laboratory Findings.-(Nov. 13, 1935.) Hemoglobin 45 per cent (Tallqv-ist); R.B.C. 1,500,000; leucocytes 36,800; neutrophiles 80 per cent; lymphocytes 20 per 667

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AMERICAN JOURNAL OF OBSTE'fRICS AND GYNECOLOGY

cent. Smear revealed anisocytosis, poikilocytosis, many sickle cells, and an occasional normoblast. Moist preparation for sickling revealed approximately 50 per cent sickle cells.. There was an increased resistance of red cells to hypotonic solutions. Icterus index 22. Serum bilirubin 1.5 mg. per 100 c.c. The Wassermann reaction was negative. Blood chemistry was within normal limits. Serum protein was 6.25 gm. per 100 c. c. with 2.60 gm. of albumin. Urinalysis: sp. gr. 1.014, albumin 3-plus, occasional pus and blood cells. Concentration and dilution tests showed maximum range of sp. gr. from 1.002 to 1.012. Phenolsulphonephthalein test (intravenous administration), 45 per cent excretion in two hours. Gastric analysis showed no free hydrochloric acid after Ewald test meal. Stool examinations were repeatedly negative . .Roentgenologic Studies.-Teleoroentgenogram of the heart showed definite cardiac enlargement. Total transverse diameter of heart was 15.2 em.; internal diameter

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of thorax was ;;]3.7 c.m. l<'ilrns of the left hand and foot revealed mottled areas of decreased density in the ends of the metacarpals, the metatarsals anti the phalanges. 'l'he midportions of the shafts of all bones appeared normal. A film of the skull showed mottled areas of decreased density throughout the vault, but chiefly in the parietal and frontal regions.

Course.-The patient was put to bed on a high protein, high caloric diet. Iron and ammonium citrate was given by mouth, and one ampule of liver extract was given intramuscularly daily. Although the patient's blood belongs in Group IV (Moss), on cross-matching 20 potential donors, no compatible one could be found. It was therefore impossible to give her a transfusion. The red count remained between 1,250,000 and 1,700,000 (see graph of blood counts). Termination of preg~ nancy was thought necessary, and hysterotomy with sterilization was selected as the method of choice. Operation was performed on Dec. 9, 1935. The postoperative

LEWIS:

SICKLE CELL ANEMIA WITH PREGNANCY

669

course was uneventful. She was kept in the hospital for observation until Jan. 25, 1936, and when dismissed she was greatly improved. The patient was readmitted

Fig. 2.-Pho tog-raph of teleoroentgenogram which shows definite cardiac hypertrophy, a t ypical fe ature of sickle cell anemia.

Fig. 3.- X-ray of skull showing mottled areas of decreased density throughout the vault.

for further study on Feb. 12, 1936. The red count at this time was 214001000 with hemoglobin of 40 per cent (Sahli). The phenolsulphonephthalein test revealed 85 per cent excretion in two hours. The concentration and dilution tests remained

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AMERICAN J OURNAL OF OBSTETRICS AND GYNECOLOGY

essentially the same. Serum albumin was 3.35 gm. per 100 c.c. There was a soft, blowing, systolic murmur at the pulmonic area. P ersistent albuminuria, Grade 2 t o 3, was present throughout the period of observation. COMMENT

This case exemplifie~ the rather common error of mistaking sickle cell anemia f or rheumatic hear t disease. In a ddition it sugg·ests an interesting line of supposition. Yater and Mollari5 mention the f act that two pregnancies in t heir patient had

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terminated as abortions. Our patient had had three abortions and no full-term pregnancies. Sickle cell anemia as an etiological agen t in habitual abortions is suggested. It is well recognized that sickle cell anemia exists in both an active and a laten t state, but the causes of exacerbations and remissions are not known. Since there was a definite remission following terminati on of pregnancy in this patient, the possibility exists that pregnancy was cau~ing an exacerbation. Since pregnancy was not allowed to continue to a spontaneous termination, f urther cases must be studied before a conclusion can be reached. The bone changes as shown by x-ray have been mentioned often.s-s These are exemplified in Figs. 2 t o 4.

JAl<'FE:

AC'I'INOMYCOTIC a:aANlJI,ES IN RETEh'
671

The question arises as to the presence and effect of renal disease in this patient. With no llistory of previous acute nephritis, a normal blood pressure throughout the period of observation, and improvement of phenolsulphonephthalein excretion with remission of the anemia, the probability is that the evidence of kidney disease is on the basis of the anemia. l'l"ephrosis is to be considered. SUMMARY

1. A case of sickle cell anemia complicated by pregnancy iR presented.

2. Sickle cell anemia as a possible cause of abortions is suggested. 3. Pregnancy as a factor in exacerbations is considered. 4. This case is published with the hope that further cases will be studied to conflrm or disprove the suppositions presented. REFERENCES

(1) Herrick, J.: Arch. Int. Med. 6: 517, 1910. (2) Anderso-n, W. W., and Ware, R. L.: J. A. M. A. 99: 902, 1932. (3) Anderson, W. W., and Ware, R. L.: Am. J. Dis. Child. 44: 1055, 1932. (4) Lash, A. F.: AM. J. 0BST. & GYNEO. 27: 79, 1934. (5) Yater, W. M., and Mollari, M.: J. A.M. A. 96: 1671, 1931. Vogt, E. C., and Diamond, L. K.: Am. J. Roentgenol. 23: 625, 1930. (7) Grinnan, G.: Am. J. Roentgenol. 34: 297, 1935. (8) LeWalil, L. T.: Radiology 18: 792, 1932. 405 }'IRST

NATIONM~ BANK BDLDI!'G

ACTINOMYCOTIC GRANCLES IN A RETENTION CYST OF THE CERVIX UTERI R. H.

JAE']'E,

M.D.,

CHICAGO, ILL.

(From the Depa1·tment of Pathology of the Coole County Hospital and the Uihlein Mernorial Laboratory of the Grant Hosp·ital) ACTINOMYCOSIS of the internal female sex organs is being reported with increasing frequency, and in the majority of the cases the lesions are found to affect the ovaries, fallopian tubes, and parametria. Most of the author~ who have reviewed the literature or have described personal observations emphasize the great resistance of the uterus to the that if the uterus does become involved it is due to the extension of the process from the parametrium (Niirnberger,l Daniel and Jl.fa vrodin12 Cornell,a and others). The actinomycosis of the ovaries, tubes, and parametria is usually secondary to an infection of the intestine, particularly of the cecum or appendix. From the intestine the infection spreads to the ovaries and parametrian tissue either retroperitoneally or intraperitoneally along pericecal or periappendiceal adhesions. Blasek4 has pointed out that the actinomycotic infection may progress while the primary lesion at tile point of entrance in the intestine heals. It requires then a very careful histologic examination in order to detect the residues of the initial lesion in the intestine. Daniel and :M:avrodin2 refer to the external genitalia as a possible port of entrance of the infection. J!'rom time to time, however, cases are described which suggest that the ray fungus may directly infect the internal female genitalia, the primary lesion being located in the uterus. \'Vith regard to this primary actinomycosis of the uterus, an observation is of interest which I have recently made and which indicates that the infection may remain dormant.