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ABDOMINAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 2  |  Page : 219-220
Rare splenic manifestations of sickle cell disease


Department of Radiodiagnosis, V.S.S. Medical College Hospital, At/PO. Burla, Sambalpur, Orissa-768017, India

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Keywords: Sickle cell, Spleen

How to cite this article:
Mohanty J, Bhagat S, Panda B B, Pappachan B. Rare splenic manifestations of sickle cell disease. Indian J Radiol Imaging 2002;12:219-20

How to cite this URL:
Mohanty J, Bhagat S, Panda B B, Pappachan B. Rare splenic manifestations of sickle cell disease. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Nov 14];12:219-20. Available from: http://www.ijri.org/text.asp?2002/12/2/219/28449
Since Western Orissa comes within the "Sickle Cell Belt" of India, Sickle Cell Disease (SCD) is quite common in our region. SCD affects many organ systems in the body and spleen is one of the commonest sites of affection. Though radiological changes in spleen are well recognized, uncommon manifestations do occur. We present two such cases.


   Clinical Presentation Top


The first patient was a twenty-two year old man who came with complaints of fever and left upper quadrant pain since three weeks. The pain was not related to food intake and there was no history of vomiting or altered bowel habits. Examination revealed moderate pallor and splenomegaly. Haemoglobin electrophoresis showed SS band and his haemoglobin level was 8.4 g/dl. Antero-posterior radiograph of abdomen showed enlarged splenic shadow containing dense amorphous calcifications. US showed densely hyperechoic spleen with strong posterior shadowing making assessment of splenic size difficult. There was also hepatomegaly.

The second patient was a thirty year old man who presented with features of acute abdomen. Examination revealed tender splenomegaly and mild pallor. Haemoglobin electrophoresis showed AS band and his haemoglobin level was 11g/dl. Antero-posterior radiograph of the abdomen didn't reveal any abnormality except for enlarged splenic shadow. US showed moderate splenomegaly with massive hypoechoic region extending from upper to lower pole and involving three-quarters of splenic parenchyma. Residual normal splenic parenchyma was seen mainly at the periphery and the splenic capsule was echogenic.


   Discussion Top


CT scan of first patient showed enlarged spleen with multiple amorphous hyperdense foci (+287 HU) suggestive of calcification affecting almost the entire spleen with sparse intervening parenchyma [Figure - 1]. Liver parenchyma appeared normal. Classically homozygous SCD patients tend to have small densely calcified spleens [1]. Splenomegaly appears in the first year of life and vascular occlusions and repetitive local infarctions tend to cause the so called autosplenectomy of spleen in homozygous disease with splenic function being lost by age of five. Pathologically the end stage spleen is small and fibrotic with marked deposition of hemosiderin and calcium. Rarely splenomegaly may persist in a homozygous patient but such spleens are also non-functional, densely fibrotic and calcified [1] which was seen in our case. Moreover clinical disappearance of the spleen does not imply atrophy since splenomegaly may recur years later in some patients during intercurrent illness [2]. The cause of splenic opacification is presumed to be predominantly due to calcium and such spleens may appear on scans with bone seeking agents [3].

Ladungoye (1971) proposed a classification of four basic patterns of splenic calcification in SCD patients [4] and McCall et al (1981) gave the relative occurance of these patterns from a study on 182 patients [5] - (i) Curvilinear Calcification, attributed to old perisplenitis, seen in 12.5% of patients and affected either parts of the spleen or occasionally the entire splenic margin. (ii) Miliary or fine punctate opacification seen in 55% of patients, probably representing siderofibrotic nodules. (iii) Thick amorphous opacification from healed splenic infarcts seen in 32% of patients and consisted of dense ill-defined areas involving the whole spleen which probably developed from the fine punctate opacification as contraction of the spleen brought these lesions together (iv) A uniform parenchymatous opacity.

CT scan of the second patient showed an enlarged spleen with an extensive hypodense lesion occupying most of the splenic parenchyma with the residual normal parenchyma visible mainly at the periphery. Thin rim of calcification was evident at the splenic margin at places [Figure - 2]. Liver appeared normal in parenchymal density. These heterozygous patients have far milder disease with fewer episodes of crisis and less severe episodes of vaso-occlusion than their homozygous counterparts. Therefore the sickle cell trait spleen continues to provide a site of potential pathology including splenic abscess, infarcts, haemorrhage and rupture unlike the auto-splenectomised organ of homozygous patients [1]. Hence splenomegaly is a common finding in this group of patients. Although infarctions do occur in periods of extreme stress, they tend to be mild. Contrary to this, in our case the infarction involved almost three-quarters of the spleen which again is an uncommon occurance in heterozygous SCD patients.

 
   References Top

1.Majid D, Fishman EK, Siegelman SS, Computed tomography of the spleen and liver in sickle cell disease AJR 1984;143: 245-249.  Back to cited text no. 1    
2.Sergeant GR. Sickle cell disease: The spleen, 1st ed, New York: Oxford University Press, 1985: 109 - 123.  Back to cited text no. 2    
3.Fischer KC, Shapiro S. Treves S, Visualisation of the spleen with a bone seeking radionuclide in a child with sickle cell anemia. Radiology 1977; 122:398.  Back to cited text no. 3    
4.Lagundoye SB, Splenic calcification and sickle cell disease in Nigerians. Trop. Geogr. Med. 1971: 23: 135-40.  Back to cited text no. 4    
5.McCall IW, Vaidya S. Sergeant GR, Splenic opacification in homozygous sickle cell disease. Clin Radiol. 1981; 32: 611-15.  Back to cited text no. 5    

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Correspondence Address:
J Mohanty
Department of Radiodiagnosis, V.S.S. Medical College Hospital, At/PO. Burla, Sambalpur, Orissa-768017
India
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Source of Support: None, Conflict of Interest: None


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