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Aplastic anemia complicating pregnancy
L. T. DORGAN, MEDICAL CORPS, ~JNITED STATIW NAVY, AND TIIEUTENAP~V H. S. MCGAUC~HEY, MEDICAL CORPS, UNTTED STATES NAVY, PORTSMOUTH, VA.
CAPTAIN
the Dqartmeat
(From DIOPATHIC
aplastic
of
United
States Navnl Hospital i
anemia
I literature contains so few trerttment has been formulated. pregnancy sufficient
Obstetrics rind Bvnecology,
is indicated as number of isolated
occurring as a complication of pregnancy is rare and the such reports that no fixed opinion as to the best method of The question of whether or not. immediate termination of the soon as the diagnosis is made can be answered only when a cases have been made available for analysis.
Mrs. C. S., a &-year-old white primigravida, was first seen in the prenatal clinic of the United States Naval Hospital, Portsmouth, Va., on June 95, 194i, in the twenty-sixth week of pregnancy. She stated that she had felt very well since becoming pregnant and had no complaints; she had not previously consulted any physician in her prkgnancy. Past his. tory revealed measles, mumps, and a tonsillectomy in childhood, no other illness nor operation. Onset of menstruation at 13 years of age, regular %-day cycle, flow of 3 days’ duration; last menstrual period Dec. 24, 194% Physical examination was entirely within normal limits, with the uterus enlarged t,o the size of a twent.ysis weeks’ gestation. The chest x-ray was normal, blood Kahn test negative, blood type A, Rh factor positive, red blood rount 4,76O,OOO, hemoglobin 13.5 Gm., urinalysis normal. Routine prenatal caatre was instituted and the patient was instructed to return in four weeks. On July 25 (thirty weeks of gestation’) a one plus pedal edema was noted, urinalysis was normal, blood pressure IOO/54. A low sodium chloride diet was prescribed t,ogether with ammonium chloride> S Gm. daily for three days, and she was instructed to return in one week. At the next visit,, August 5 (32 weeks of gestation) the tract of pedal cdema was si,ill present, complexion appearecl somewhat sallow~ and there were several small asymptomatic purpuric spots on the right ralf; the patient, could not recall having bruised herself in any may. Blood pressure was IlO/FO, pulse X4, urinalysis normal, a tourniquet test showed normal capillary fragility. Blood studies revealed the follon;ing : red blood cells 3,470,000, hemoglobin 10 Gm., platelets 83,000, white hIood c*c?lls 3,500, wit,h normal distrihnt,ion in the clotting time t.hree minutes. differential count, bleeding lime one minute forty-five seconds, The patient was placed on inrreasing daily amounts ol liver extract. iron, and multiple vitamins and referred to the abnormal obstetrical rlinic for further study of the thromho cytopenia and leueopenia. During the next four weeks she was symptom free, the pu~puri~ spots faded, ant1 no new ones appeared. Further blood studies were made with the following findings: retieulocyte count 3 per rent, t,olor index 1.1, volume index 1 2, hematoerit 30 per cent, prothrombin time 135 per cent, red cell fragilit,y normal, capiliary fragility normal. On September 19 (38 weeks of gestation) a profound change was noted in hor conrlition; pallor was marked, mucous membranes were pale, the pulse rate was 110. She coniplained of marked fatigability, palpitation, giddiness, a tendency to bruise easily, and bleeding from the gums. Further questioning revealed that she was a graduate nurse although she had done no nursing for several years, there had been no contact with any type of radiation or noxious agent, no drugs had been taken other than the medication prescribed in the clinic (liver extract, iron, vitamins, and ammonium chloride), and there was no evidence of any abnormal bleeding tendenry in eit,her her or her family’s past histor?. She was immediately admitted to the hospital for study and therapy.
*The opinions expressed herein are those of the authors and do not necessarily represent those of the Naval Service as a whole.
Volume 61 Numbs
6
APLASTIC
ANEMIA
COMPLICATING
PREGNANCY
1391
On admission the patient appeared pallid but otherwise not acutely ill. Blood pressure was 110/70, pulse 100, respirations 24, temperature 99.4” F. The skin was of normal texture but pale and there were several small purpuric spots on the left forearm. Eyegrounds were normal except for retinal pallor. A few small discrete lymph nodes were palpable in the anterior cervical chain but there was no generalized adenopathy. Neither the liver nor spleen was palpable. The uterus was enlarged to the size of a 35 weeks’ gestation with the fetus Fetal heart toues were clear and regular. A one lying in a right occiput anterior position. Laboratory studies at the time were as follows: red plus dependent edema was present. I6 per cent, white blood cells blood cells 1,770,000, hemoglobin below 7.5 Gm., hematocrit 4,700, with 2 band forms, 44 segmented forms, and 54 lymphocytes, reticulocyte count 0.05 per cent., ieterie index 8, clot retraction very poor, blood sedimentation rate 31 mm. per hour, total serum proteins 5.8 mg. per cent, albumin-globulin ratio 1.32J1.0, cephalin flocculation negative, gastric analysis, no free hydrochloric acid and 18 total acids, urinalysis normal. A sternal puncture was performed and the marrow reported as showing a depression of all elements, the picture being consistent with a diagnosis of aplastic anemia. high protein-carbohydrateTherapy consisted of multiple whole blood transfusions, vitamin diet, liver extract, iron, yellow bone marrow capsules, and intramuscular Pentonueleotide. The blood count rose and she felt greatly improved; on September 29, ten days At this time the red blood count after admission, she entered labor spontaneously at term. was 3,050,000, hemoglobin 10.0 grams. One thousand C.C. of whole blood were crossmatched and made available for immediate use. The course of labor was uneventful and after fifteen hours she was delivered with low forceps through a mediolateral episiotomy of a living male infant, with open-drop ether anesthesia. The infant weighed 6 pounds, 15 ounces, breathed and cried spontaneously, and appeared normal. The third stage was normal with an estimated blood 1033 of 300 C.C. A whole-blood transfusion of 500 C.C. was given and the patient appeared to be in excellent condition. Following delivery all therapy was continued and for the first five days she was afebrile, the lochia normal, breasts engorged, corpus involuting well, red blood count 3,600,OOO. On the sixth postpartum day she became febrile, the temperature rising and falling in a septic manner thereafter to a daily high of 105’ F. Physical examination revealed slight vaginal bleeding Lochial culture and blood and a uterus slightly larger than expected for the puerperium. culture were: negative. Penicillin and streptomycin were given in therapeutic and later massive doses without any noticeable effect on the septic course. The vaginal bleeding gradually increased and occult blood was present in the gastric content and in the stools. The red blood count showed a gradual fall and 500 cc. of whole blood were required approximately every 48 hours to maintain the count above three million. The uterine bleeding continued and on the seventeenth postpartum day the uterus was explored digitally and with a curette. No tissue was found, and the cavity was packed with gauze. Blood continued to ooze about the pack and bleeding recurred upon its removal twenty-four hours later. By the twenty-seventh postpartum day the loss of blood per vaginam was faster than replacement could be made. It was felt that a rapid supravaginal hysterectomy should be attempted as a last resort measure and on October 26 the twenty-seventh postpartum day, this was performed. The procedure was complicated by the edema and friability of all of the pelvic tissues which caused ligatures to cut out but was accomplished in a short period of time; the patient withstood the operation well although her condition was critical at the time despite massive infusions of whole blood. Grossly the uterus was about twice normal size, pale in color, and showed a spongy edema of the musculature. Microscopic sections revealed subinvolution and edema of the myometrium,, the endometrium was scanty, and the glands in an early proliferative phase. Following the surgery the patient responded well for about forty-eight hours although the temperature continued its daily rise to 105” F. On the third postoperative day she began to bleed profusely from the nose, mouth, and gastrointestinal tract, and died on the fourth postoperative day with clinical findings of myocardial failure and pulmonary edema.
DORGAN
1392
AND
MC GAUGHEY
At autopsy the following pertinent findings were> rec.orded: multiple ecchymotic spots diffusely spread over the entire body; petechial and ecchymotic hemorrhages of all visceral and parietal surfaces; edema of the lungs ; adhesive peritonitis at the site of the recent pelvic surgery; hemorrhagic gastroenteroeolitis; enlargement of the aortic, mesenterir, and lumbar lymph nodes, The liver appeared normal grossly and on its cut surfare. The spleen was moderately enlarged, weight 233 grams, color deep red; its cut surface appeared normal except for congestion. Microscopic sections of vertebral body ant1 rib marrow showed marked and a great increase in the number of plasma hypoplasia with an increase of fatty tissue, and r&iculohistiocytie cells. There appeared to be a grentrr depression of the myeloid than of the erythroid elements. Pntlrological
diagnosis:
Aplastic
anemia.
The infant was discharged from the> hospital ounce, physical examination including hlooll studies
at 1 rnontlt of cntirely normal.
age,
weight
9 pounds,
I
CAPTAIN
the Dqartmeat
(From DIOPATHIC
aplastic
of
United
States Navnl Hospital i
anemia
I literature contains so few trerttment has been formulated. pregnancy sufficient
Obstetrics rind Bvnecology,
is indicated as number of isolated
occurring as a complication of pregnancy is rare and the such reports that no fixed opinion as to the best method of The question of whether or not. immediate termination of the soon as the diagnosis is made can be answered only when a cases have been made available for analysis.
Mrs. C. S., a &-year-old white primigravida, was first seen in the prenatal clinic of the United States Naval Hospital, Portsmouth, Va., on June 95, 194i, in the twenty-sixth week of pregnancy. She stated that she had felt very well since becoming pregnant and had no complaints; she had not previously consulted any physician in her prkgnancy. Past his. tory revealed measles, mumps, and a tonsillectomy in childhood, no other illness nor operation. Onset of menstruation at 13 years of age, regular %-day cycle, flow of 3 days’ duration; last menstrual period Dec. 24, 194% Physical examination was entirely within normal limits, with the uterus enlarged t,o the size of a twent.ysis weeks’ gestation. The chest x-ray was normal, blood Kahn test negative, blood type A, Rh factor positive, red blood rount 4,76O,OOO, hemoglobin 13.5 Gm., urinalysis normal. Routine prenatal caatre was instituted and the patient was instructed to return in four weeks. On July 25 (thirty weeks of gestation’) a one plus pedal edema was noted, urinalysis was normal, blood pressure IOO/54. A low sodium chloride diet was prescribed t,ogether with ammonium chloride> S Gm. daily for three days, and she was instructed to return in one week. At the next visit,, August 5 (32 weeks of gestation) the tract of pedal cdema was si,ill present, complexion appearecl somewhat sallow~ and there were several small asymptomatic purpuric spots on the right ralf; the patient, could not recall having bruised herself in any may. Blood pressure was IlO/FO, pulse X4, urinalysis normal, a tourniquet test showed normal capillary fragility. Blood studies revealed the follon;ing : red blood cells 3,470,000, hemoglobin 10 Gm., platelets 83,000, white hIood c*c?lls 3,500, wit,h normal distrihnt,ion in the clotting time t.hree minutes. differential count, bleeding lime one minute forty-five seconds, The patient was placed on inrreasing daily amounts ol liver extract. iron, and multiple vitamins and referred to the abnormal obstetrical rlinic for further study of the thromho cytopenia and leueopenia. During the next four weeks she was symptom free, the pu~puri~ spots faded, ant1 no new ones appeared. Further blood studies were made with the following findings: retieulocyte count 3 per rent, t,olor index 1.1, volume index 1 2, hematoerit 30 per cent, prothrombin time 135 per cent, red cell fragilit,y normal, capiliary fragility normal. On September 19 (38 weeks of gestation) a profound change was noted in hor conrlition; pallor was marked, mucous membranes were pale, the pulse rate was 110. She coniplained of marked fatigability, palpitation, giddiness, a tendency to bruise easily, and bleeding from the gums. Further questioning revealed that she was a graduate nurse although she had done no nursing for several years, there had been no contact with any type of radiation or noxious agent, no drugs had been taken other than the medication prescribed in the clinic (liver extract, iron, vitamins, and ammonium chloride), and there was no evidence of any abnormal bleeding tendenry in eit,her her or her family’s past histor?. She was immediately admitted to the hospital for study and therapy.
*The opinions expressed herein are those of the authors and do not necessarily represent those of the Naval Service as a whole.
Volume 61 Numbs
6
APLASTIC
ANEMIA
COMPLICATING
PREGNANCY
1391
On admission the patient appeared pallid but otherwise not acutely ill. Blood pressure was 110/70, pulse 100, respirations 24, temperature 99.4” F. The skin was of normal texture but pale and there were several small purpuric spots on the left forearm. Eyegrounds were normal except for retinal pallor. A few small discrete lymph nodes were palpable in the anterior cervical chain but there was no generalized adenopathy. Neither the liver nor spleen was palpable. The uterus was enlarged to the size of a 35 weeks’ gestation with the fetus Fetal heart toues were clear and regular. A one lying in a right occiput anterior position. Laboratory studies at the time were as follows: red plus dependent edema was present. I6 per cent, white blood cells blood cells 1,770,000, hemoglobin below 7.5 Gm., hematocrit 4,700, with 2 band forms, 44 segmented forms, and 54 lymphocytes, reticulocyte count 0.05 per cent., ieterie index 8, clot retraction very poor, blood sedimentation rate 31 mm. per hour, total serum proteins 5.8 mg. per cent, albumin-globulin ratio 1.32J1.0, cephalin flocculation negative, gastric analysis, no free hydrochloric acid and 18 total acids, urinalysis normal. A sternal puncture was performed and the marrow reported as showing a depression of all elements, the picture being consistent with a diagnosis of aplastic anemia. high protein-carbohydrateTherapy consisted of multiple whole blood transfusions, vitamin diet, liver extract, iron, yellow bone marrow capsules, and intramuscular Pentonueleotide. The blood count rose and she felt greatly improved; on September 29, ten days At this time the red blood count after admission, she entered labor spontaneously at term. was 3,050,000, hemoglobin 10.0 grams. One thousand C.C. of whole blood were crossmatched and made available for immediate use. The course of labor was uneventful and after fifteen hours she was delivered with low forceps through a mediolateral episiotomy of a living male infant, with open-drop ether anesthesia. The infant weighed 6 pounds, 15 ounces, breathed and cried spontaneously, and appeared normal. The third stage was normal with an estimated blood 1033 of 300 C.C. A whole-blood transfusion of 500 C.C. was given and the patient appeared to be in excellent condition. Following delivery all therapy was continued and for the first five days she was afebrile, the lochia normal, breasts engorged, corpus involuting well, red blood count 3,600,OOO. On the sixth postpartum day she became febrile, the temperature rising and falling in a septic manner thereafter to a daily high of 105’ F. Physical examination revealed slight vaginal bleeding Lochial culture and blood and a uterus slightly larger than expected for the puerperium. culture were: negative. Penicillin and streptomycin were given in therapeutic and later massive doses without any noticeable effect on the septic course. The vaginal bleeding gradually increased and occult blood was present in the gastric content and in the stools. The red blood count showed a gradual fall and 500 cc. of whole blood were required approximately every 48 hours to maintain the count above three million. The uterine bleeding continued and on the seventeenth postpartum day the uterus was explored digitally and with a curette. No tissue was found, and the cavity was packed with gauze. Blood continued to ooze about the pack and bleeding recurred upon its removal twenty-four hours later. By the twenty-seventh postpartum day the loss of blood per vaginam was faster than replacement could be made. It was felt that a rapid supravaginal hysterectomy should be attempted as a last resort measure and on October 26 the twenty-seventh postpartum day, this was performed. The procedure was complicated by the edema and friability of all of the pelvic tissues which caused ligatures to cut out but was accomplished in a short period of time; the patient withstood the operation well although her condition was critical at the time despite massive infusions of whole blood. Grossly the uterus was about twice normal size, pale in color, and showed a spongy edema of the musculature. Microscopic sections revealed subinvolution and edema of the myometrium,, the endometrium was scanty, and the glands in an early proliferative phase. Following the surgery the patient responded well for about forty-eight hours although the temperature continued its daily rise to 105” F. On the third postoperative day she began to bleed profusely from the nose, mouth, and gastrointestinal tract, and died on the fourth postoperative day with clinical findings of myocardial failure and pulmonary edema.
DORGAN
1392
AND
MC GAUGHEY
At autopsy the following pertinent findings were> rec.orded: multiple ecchymotic spots diffusely spread over the entire body; petechial and ecchymotic hemorrhages of all visceral and parietal surfaces; edema of the lungs ; adhesive peritonitis at the site of the recent pelvic surgery; hemorrhagic gastroenteroeolitis; enlargement of the aortic, mesenterir, and lumbar lymph nodes, The liver appeared normal grossly and on its cut surfare. The spleen was moderately enlarged, weight 233 grams, color deep red; its cut surface appeared normal except for congestion. Microscopic sections of vertebral body ant1 rib marrow showed marked and a great increase in the number of plasma hypoplasia with an increase of fatty tissue, and r&iculohistiocytie cells. There appeared to be a grentrr depression of the myeloid than of the erythroid elements. Pntlrological
diagnosis:
Aplastic
anemia.
The infant was discharged from the> hospital ounce, physical examination including hlooll studies
at 1 rnontlt of cntirely normal.
age,
weight
9 pounds,
I