Isolated spleen agenesis: a rare cause of thrombocytosis mimicking essential thrombocythemia.
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Thrombocytosis is a common feature of myeloproliferative disorders but may also result from various conditions including chronic iron deficiency, hemorrhage, chronic inflammation and splenectomy. We report two cases of secondary thrombocytosis caused by isolated and congenital asplenia, mimicking essential thrombocythemia. These two adult cases of spleen agenesis were unexpected. We conclude that in thrombocytosis without clinical evidence of splenomegaly, attentive screening of blood in search of Howell-Jolly bodies and abdominal ultrasonography should always be performed not only to detect mild spleen enlargement but also to make sure of the presence of this organ. (+info)
A new method for studying splenic reticuloendothelial dysfunction in sickle cell disease patients and its clinical application: a brief report.
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Differential interference contrast (DIC) microscopy (Nomarsky optics) readily demonstrates the formation of "pits" or crater-like depressions in red cell membranes of splenectomized individuals. Splenic reticuloendothelial dysfunction characteristic of many patients with sickle cell disease (SCD) can be demonstrated by technetium spleen scans, but this technique is expensive, requires injection of radioactive material into children, and is cumbersome to perform at regular intervals. However, pit formation in red cells, which also appears to reflect splenic dysfunction, can readily be quantitated in a finger-stick blood sample using DIC microscopy. In this study, the degree of red cell pitting was compared with results of technetium spleen scans and measurements of Howell-Jolly bodies in individuals with sickle cell disease. The average pitted cell percentage in the control population was 0.5% +/- 0.5 (range 0.0-2.6) and 30.5% +/- 13.9 in the SCD population (range 2.4-71.1) (less than 0.001). Of the individuals studied with SCD, 12 also had technetium (99mTc) sulfur colloid scans and measurements of Howell-Jolly bodies. The percentage of Howell-Jolly bodies was low and did not correlate well with the degree of splenic visualization. However, there was an excellent correlation between pit count and splenic dysfunction as measured by spleen scan. Determination of red cell pitting, therefore, appears to offer a simple means for clinical evaluation of splenic reticuloendothelial function in patients with SCD. (+info)
A multicenter trial of the effectiveness of zeta-globin enzyme-linked immunosorbent assay and hemoglobin H inclusion body screening for the detection of alpha0-thalassemia trait.
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Molecular basis for dominantly inherited inclusion body beta-thalassemia.
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Analysis of the molecular basis of dominantly inherited beta-thalassemia in four families has revealed different mutations involving exon 3 of the beta-globin gene. It is suggested that the phenotypic difference between this condition and the more common recessive forms of beta-thalassemia lies mainly in the length and stability of the abnormal translation products that are synthesized and, in particular, whether they are capable of binding heme and producing aggregations that are relatively resistant to proteolytic degradation. (+info)
Prevalence of Howell-Jolly bodies caused by partial splenic embolization for portal hypertension.
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OBJECTIVE: Postsplenectomy sepsis (PSS) and overwhelming postsplenectomy infection (OPSI) following splenectomy or the development of hyposplenism are associated with a high mortality rate. The presence of Howell-Jolly bodies (HJBs) in peripheral erythrocytes is attracting attention as a parameter of hyposplenism. To date, whether HJBs appear following partial splenic embolization (PSE) has not been investigated. Therefore, we examined the prevalence of HJBs in patients who have undergone PSE. METHODS: Whether HJBs were present in 95 patients who underwent PSE between November 2007 and August 2012 was assessed. RESULTS: No serious complications occurred due to PSE; however, 17 of the 95 patients (17.89%) exhibited HJBs during the follow-up. The residual spleen volume and splenic infarction rate did not differ significantly compared to those observed in the HJB-negative group. CONCLUSION: With the recent increase in the use of autoanalyzers, the opportunities to perform microscopic examinations have been decreasing. Therefore, the presence of HJBs, which can only be confirmed visually, may be overlooked, and the clinical significance of these bodies tends to be disregarded. However, the presence of HJBs is associated with a risk of PSS and OPSI due to hyposplenism. Because HJBs are common in the peripheral erythrocytes of patients who have undergone PSE, irrespective of the residual spleen volume or splenic infarction rate, the presence or absence of HJBs should be assessed visually. In HJB-positive patients, preventing serious infections, for example, by administering the pneumococcal vaccine, is important. (+info)
Reversible functional asplenia in chronic aggressive hepatitis.
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A 61-year-old man presented with aggressive hepatitis. Howell-Jolly bodies were present in circulating erythrocytes and the spleen failed to accumulate intravenously administered Tc-99 m sulfur colloid. The patient thus demonstrated functional asplenia. He was treated with high doses of steroids. Four years later, Howell-Jolly bodies were no longer present in circulating erythrocytes. In addition, the spleen had regained the ability to accumulate intravenously injected radiocolloid. Hence, the patient had reversed his functional asplenia. The reported cases of this disorder (reversible functional asplenia) were reviewed and a preliminary classification was proposed. (+info)