Pulmonary infarction in sickle cell trait.

A young black man presented with unexplained pleuritic chest pain. A hematologic evaluation revealed sickle cell trait, Chest roentgenograms, ventilation/perfusion lung scanning and a pleural-parenchymal lung biopsy documented pulmonary infarction. Sickle cell trait with resultant pulmonary infarction should be considered in black subjects with unexplained pulmonary diseases.     

Non hypoxia-related splenic infarct in a patient with sickle cell trait and infectious mononucleosis

Splenic infarction in patients with sickle cell trait is usually related to hypoxic conditions, while non-hypoxia-related infarcts are extremely rare. We report on a case of a 17-year-old male patient, living at sea level, who developed a severe left upper quadrant abdominal pain during the course of a febrile episode. On physical examination he had a mildly palpable but extremely painful spleen. A spleen scan revealed 2 areas of impaired radionucleide distribution. Hepatic enzymes were moderately increased and the IgM anti-EBV antibodies positive. Hemoglobin electrophoresis revealed the presence of 42% of hemoglobin S. A probable diagnosis of splenic infarction was established in a patient with sickle cell trait, during the course of infectious mononucleosis. The patient was treated symptomatically. The conditions of splenic congestion induced by the EBV infection and the high-grade fever may have contributed to splenic sequestration and subsequent infarcts.     

Pulmonary function in sickle cell trait

Pulmonary function abnormalities, which have been reported to occur in persons with sickle cell trait (hemoglobin AS), could intensify the hypoxic stimulus for the systemic formation of sickle cells at high altitude. We sought to determine whether pulmonary function abnormalities occur as a result of exposure to high altitudes in persons with hemoglobin AS. In a prospective study, 13 men with hemoglobin AS ("cases") and 13 controls (hemoglobin AA) matched by age, sex, and race were exposed to five to seven altitude simulations (ranging from 1524 to 7620 m [5000 to 25,000 ft]) in a hypobaric chamber. Measurements of diffusing capacity for carbon monoxide (DLco), forced vital capacity, forced expiratory volume in one second, and forced midexpiratory flow were obtained before and after each exposure. Data before exposures did not differ statistically between cases and controls. Altitude had no systematic effect on DLco or spirometric values in cases compared with values in controls (p greater than 0.05). Individual declines in forced vital capacity or DLco of more than 10% occurred with similar frequency in both groups. Measurements made after the series of exposures showed no change from those made before. We conclude that short serial exposures to hypoxia at high altitudes does not acutely or cumulatively alter DLco or spirometric values in healthy, nonexercising persons with sickle cell trait.     

Splenic infarction associated with sickle cell trait at low altitude

Abstract

Objective and importance

Sickle cell trait is widely known to be associated with splenic infarction at high altitudes. Although textbooks and reviews imply that this complication does not occur at low altitudes, we encountered such a case and identified several previous cases in the literature.

Clinical presentation

An 18-year-old woman with sickle cell trait who resided near sea level presented with left upper quadrant abdominal pain and was found to have multiple splenic infarcts. She was otherwise well, with no comorbidities that would predispose to hypoxemia or vascular injury. A review of the literature revealed 12 previously published cases of low-altitude splenic infarction in patients with sickle trait; 7 of those patients had comorbidities that likely predisposed to splenic infarction.

Intervention

None.

Conclusion

Spontaneous splenic infarction can occur in patients with sickle trait who live at low altitudes. It is unclear whether this complication is rare, or whether it is relatively common but under-recognized.     

Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration

The association of sickle cell trait (SCT) and hereditary spherocytosis (HS) has been reported in only 18 patients. Three of these 18 patients experienced splenic infarct or acute splenic sequestration. We report here a 46-year-old African-American male, the oldest reported case to date, who experienced episodes of hemolysis and severe left upper quadrant pain for the past 26 years. The patient had compensated hemolysis with splenomegaly. A CT scan of the abdomen revealed a large infarct in the spleen. The diagnosis of SCT was confirmed with isoelectric focusing, cation exchange and reverse-phase HPLC. The presence of a silent, interacting globin variant as the cause of hemolysis and sickling in the spleen was ruled out by sequencing of the alpha1-, alpha2- and beta-globin genes. The diagnosis of HS was established by an osmotic fragility test. The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case.     

Massive splenic infarction in Saudi patients with sickle cell anemia: a unique manifestation

Splenic infarcts are common in patients with sickle cell anemia (SCA), but these are usually small and repetitive, leading ultimately to autosplenectomy. Massive splenic infarcts on the other hand are extremely rare. This is a report of our experience with 8 (4 males and 4 females) cases of massive splenic infarction in patients with SCA. Their ages ranged from 16 to 36 years (mean 22 years). Three presented with left upper quadrant abdominal pain and massive splenic infarction on admission, while the other 5 developed massive splenic infarction while in hospital. In 5 the precipitating factors were high altitude, postoperative, postpartum, salmonella septicemia, and strenuous exercise in one each, while the remaining 3 had severe generalized vasoocclusive crises. Although both ultrasound and CT scan of the abdomen were of diagnostic value, we found CT scan more accurate in delineating the size of infarction. All our patients were managed conservatively with I.V. fluids, analgesia, and blood transfusion when necessary. Diagnostic aspiration under ultrasound guidance was necessary in two patients to differentiate between massive splenic infarction and splenic abscess. Two patients required splenectomy during the same admission because of suspicion of secondary infection and abscess formation, while a third patient had splenectomy 2 months after the attack because of persistent left upper quadrant abdominal pain. In all the 3 histology of the spleen showed congestive splenomegaly with massive infarction. All of our patients survived. Two patients subsequently developed autosplenectomy while the remaining 3 continue to have persistent but asymptomatic splenomegaly. Massive splenic infarction is a rare and unique complication of SCA in the Eastern Province of Saudi Arabia, and for early diagnosis and treatment, physicians caring for these patients should be aware of such a complication.     

Bacteroides (Parabacteroides) distasonis splenic abscess in a sickle cell patient

Splenic abscess is not an uncommon complication of patients with sickle-cell disease. Here we describe an 18 year-old boy with sickle cell disease and left upper quadrant abdominal pain. Computerized axial tomography revealed left sided free flowing pleural effusion and splenomegaly with liquefaction and possible gas formation. The splenic fluid grew an unusual organism known as Bacteroides distasonis. The patient received antimicrobial therapy and underwent a splenectomy with full recovery. The spleen was cystically infarcted and measured 22 x 16 x 5 cm. The capsule was thickened and covered by fibrinous exudate. Histopathologic examination of the spleen showed complete necrosis with reparative fibrosis. This case presents an unusual cause of splenic abscess due to Bacteroides distasonis with a subacute to chronic course. The presence of fever and left sided pleuritic chest pain in patients with sickle cell disease should raise the suspicion of splenic abscess.     

Pulmonary embolism in a patient with Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital disorder characterized by cutaneous capillary malformations, venous-lymphatic anomalies, hypertrophy of soft tissue and bone in the area of increased vascularity and arteriovenous fistulas with shunting. In this article we report the case of a 25 year old man with KTWS who had multiple surgical operations because of arteriovenous malformations. He admitted with pulmonary embolism attack to the hospital although he was taking prophylactic heparin treatment. We evaluate the risk of pulmonary embolism in patients with KTWS, being aware of the risk of deep venous thrombosis in progress of the disease and the importance of early diagnosis in prognosis.     

Pulmonary embolism in a patient with pernicious anemia and hyperhomocysteinemia

We report the case of a 60-year-old woman with a history of ataxia who sought evaluation after a syncopal episode. A diagnostic workup revealed pulmonary emboli, pernicious anemia (PA), hyperhomocysteinemia, and a G20210A prothrombin gene mutation. She was successfully treated with homocysteine-lowering therapy, including high doses of oral cobalamin. She also received oral anticoagulation for 6 months. At 1 year of follow-up, no further thrombotic episodes had occurred. Our report highlights the thrombotic risk of hyperhomocysteinemia secondary to PA in a patient with the G20210A prothrombin gene mutation.