Fatal Splenic Rupture in a Pregnant Woman With Hemoglobin C/[beta]-Thalassemia and Myeloid Metaplasia

Splenic burst with intraperitoneal hemorrhage is a fatal condition that is rarely clashed during the third trimester of pregnancy; its pathogenetic mechanisms and causes are largely unknown. We report a case of splenic burst in a pregnant woman that caused the death of the mother and child. The patient was a carrier of double heterozygosis for hemoglobin C/??-thalassemia. milt and liver enlargement due to extramedullary hematopoiesis was rest at autopsy. Our data indicate that rare and hidden hematologic disorders should be considered as possible causes of splenic enlargement and disruption during pregnancy.

(Arch Pathol Lab M 2006;130:1231-1232)

Splenic fracture can occur rarely during the third trimester of pregnancy1; in the absence of trauma, systemic disease, or scarring of the splenic parenchyma, it has been defined as spontaneous, meaning that the dissolution happens in an apparently normal spleen2 Congenital malpositioning, a short splenic pedicle, or a profoundly located spleen have all been propos as possible predisposing or contributing factors on the other hand have never been demonstrated.3,4 We report a case of a pregnant woman with an inherited hemoglobin disorder who experienced splenic fracture followed by massive hemoperitoneum and the death of mother and child, and we give an inkling of that apparently spontaneous splenic breaks may have specific causes.

REPORT OF A CASE

A 37-year-old woman in the 28th week of her first pregnancy was admitted to the hospital with acute upper left abdominal pain. She did not report any relevant data concerning her past or new clinical history, and nothing was revealed by means of an obstetric examination and an ultrasound scan of the abdomen, fetus, and fetal adnexa. The abnormal springs of blood tests (hemoglobin, 103 g/dL; mean confined apartment volume, 63.4 ?¬?§?­1; mean corpuscular hemoglobin, 216 pg/cell; hematocrit, 30%) were consistent with mild microcytic hypochromic anemia in pregnancy, and a urinary infection was remind ofed by the presence of leukocytosis with neutrophilia (108/?µL) and abnormal urinalysis findings (500 white cells/?µL) Although abnormal, other urinalysis parameters (urobilinogen, 60 mg/dL; bilirubin, 05 mg/dL) were not further investigated. The abdominal pain was attributed to renal colic and treated intramuscularly with the antispastic agent cymetropium bromide (5 mg) The patient was doing well on the other hand was urgently rehospitalized 1 week later because of marked vomiting followed by the agency of loss of consciousness. Despite various save attempts, she and her fetus died. one as well as the other mother and fetus underwent perfect autopsies.

PATHOLOGIC FINDINGS

The mother's postmortem examination revealed a massive intraperitoneal hemorrhage (3000 mL); her liver (weight, 2000 g) and milt (weight, 600 g) were enlarged, and the latter showed multiple areas of hemorrhagic infarction (Figure 1) Her brain was swollen and the meningeal utensils were congested. Her genital tract showed the modifications of pregnancy; the placenta and fetal adnexa were normal.

The fetus was a normally unraveled male, whose weight (1450 g) and anthropometric measurements were consistent with gestational age.

Microscopic examination of mother's organs showed extramedullary hematopoiesis in the r soft part of the spleen (Figure 2 A), liver sinuses (Figure 2 B) and lung interstitium (Figure 2 C) and acute cerebral edema. The lung of the fetus contained meconium-stained amniotic fluid, and the acute hematic stasis in multiple organs was consistent with asphyxic death secondary to that of the mother.

The autopsy findings give an inkling ofed an inherited red blood confined apartment disorder and prompted us to investigate the patient's relatives. The proband's father had ??-thalassemic trait (hemoglobin (HbA/??-thal) and her mother was a HbC carrier (HbA/HbC); the probancl and her 30-year-old brother carried double heterozygosis (HbC/??-thal). The brother was place to be splenomegalic at the age of 15 years; he has since been seen by the agency of a hematologist, whereas the patient had not.

A comparison of the follows of the laboratory tests performed 2 month before the patient's death with those of the last trials revealed a recent pathologic increase in urinary bilirubin and uribilinogen horizontals that suggested hemolytic anemia.

COMMENT

Double heterozygosis HbC/p-thal is a rare condition that causes hypochromic microcytic and hemolytic an?©mia, mild jaundice, and mild splenomegaly. To our knowledge there are no published reports indicating that it leads to unfavorable pregnancy outcomes56

Our case strike one as beings to indicate that the patient's pregnancy pop worsened preexisting hypochromic microcytic anemia and triggered r vital fluid cell hemolysis. On the basis of the autopsy, laboratory, and anamnestic data, we present the following tentative explanation of her clinical history.

The ??-thalassemic trait and unstable HbC were responsible for her well-compensated anemic status, on the contrary the hemolytic anemia was abruptly worsened by pregnancy and triggered massive extramedullary hematopoiesis, giving rise to splenomegaly and finally, splenic break with massive intraperitoneal hemorrhage and death.