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Year : 2009 | Volume
: 52
| Issue : 3 | Page : 448-450 |
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Idiopathic splenic arteriolar hyalinosis |
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Jyotsna Madan1, Ashish K Mandal2
1 Department of Pathology, Santosh Medical College and Hospital, Ghaziabad, India 2 Department of Pathology, Maulana Azad Medical College, New Delhi, India
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Date of Web Publication | 12-Aug-2009 |
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How to cite this article: Madan J, Mandal AK. Idiopathic splenic arteriolar hyalinosis. Indian J Pathol Microbiol 2009;52:448-50 |
Sir,
Hyalinization of arterioles is a change which is observed microscopically in several organs in the body like kidney, pancreas and skin etc, and caused by various clinical conditions like diabetic microangiopathy, thrombotic thrombocytopenic purpura (TTP), idiopathic thrombocytopenic purpura (ITP), idiopathic portal hypertension and systemic lupus erythematosus (SLE). [1],[2],[3],[4] The hyalinization of arteries and arterioles appears as concentric layers or onion skin lesion of media. We report an idiopathic variety of this interesting and rare histological change in spleen, which most often is diagnosed as tuberculosis or granulomatous lesion leading to splenectomy.
A 45-year-old female presented with left hypochondrium pain and fullness for the past one and a half years. Patient was a known case of systemic hypertension taking irregular treatment for five years. At the time of admission, her blood pressure was 160/100 mm of Hg. Clinical examination revealed just palpable spleen with tenderness. No signs of portal hypertension could be identified. She had no history of fever, jaundice, or bleeding tendencies. Hematological investigation showed a hemoglobin of 10.4 gm% and erythrocyte sedimentation rate of 45 mm in the first hour. Prothrombin time was within normal limits. Widal test, rheumatoid arthritis factor and antinuclear antibodies were negative. All other biochemical profiles were within normal limits.
A 45-year-old female was admitted with complaints of pyrexia of unknown origin. She was suffering from fever (100 F) for six months. There was no history of evening rise of temperature or chills and rigors. Abdominal examination revealed mild splenomegaly. Rest of the systemic examination was within normal limits. There was no evidence of portal hypertension. Hematological investigation showed high erythrocyte sedimentation rate of 35 mm of Hg in the first hour. Antinuclear antibodies and double-stranded DNA antibodies were also negative.
Ultrasound of abdomen revealed multiple speckled/punctate calcification of spleen. Thus a clinical diagnosis of calcific tuberculous lesion was considered and splenectomy was done to confirm the diagnosis in both the cases.
Grossly, the spleens were grayish in color and weighed 120 g (Case 1) and 100 g (Case 2). The capsule was smooth and no irregularity was seen. Cut surface revealed prominent, small, multiple, grey-white, firm nodules varying in measurement from < 0.1 to 0.3 cm [Figure 1].
Microscopically, the spleen showed mild to moderate congestion. Lymphoid follicles were slightly enlarged. The follicular and interfollicular capillaries showed thickening and concentric hypertrophy of the media, resembling onion skin appearance [Figure 2]. The hypertrophic medial muscle was hyalinized and brightly eosinophilic in color. The lumen in most of these vessels was narrowed or obliterated. Multiple sections failed to reveal any necrosis, calcification or granuloma. Thus a final diagnosis of idiopathic splenic arteriolar hyalinosis was made. The post-splenectomy period was uneventful.
Hyalinization of arterioles of various organs like kidney, pancreas, and skin is well documented in different disease entities like SLE, diabetic microangiopathy, TTP, portal hypertension, and chronic ITP. [1],[2],[3],[4] Splenic vascular hyalinosis in the form of homogenous, eosinophilic hyaline thickening of media may develop with increasing age as a senile change.[5] Such a typical lesion in the spleen had long been considered a characteristic feature of SLE.[6] However, in our cases clinical history and laboratory findings did not support the diagnosis of SLE or any other immune disease.
Uniform, homogenous deposits of hyaline material in splenic arterioles and arteries may also be seen in patients with hypertensive and arteriosclerotic heart disease [7] and these hyaline deposits are probably composed of filtered plasma proteins that get trapped in increasing amounts in the media giving the appearance of hyaline masses. Chang et al . [4] in 1993 described splenic arteriolar hyalinosis in cases of idiopathic thrombocytopenic purpura. They reported that along with other features of ITP like follicular hyperplasia or proliferation of macrophages or both, arteriolar hyalinosis was also seen in spleens. Saracco et al . [2] studied 10 spleens and two accessory spleens of thrombotic thrombocytopenic purpura and hyalinosis was observed in 58% of cases.
In our cases numerous sections were taken which did not reveal the presence of any granuloma. The special stain for amyloid, fungus, and tubercle bacilli were negative. Clinical and laboratory data excluded the diagnosis of SLE, ITP, TTP and diabetic microangiopathy. The middle age of the patients also excluded the possibility of senile change. Thus, we conclude that these were cases of idiopathic splenic arteriolar hyalinosis. An awareness of such changes is important to prevent unnecessary splenectomy.
References | |  |
1. | Saltyakov BB, Malkina LA. Vascular hyalinosis in diabetic microangiopathy. Arch Pathol 1975;3:38-44. |
2. | Saracco SM, Farhi DC. Splenic pathology in thrombotic thrombocytopenic purpura. Am J Surg Pathol 1990;14:223-9. [PUBMED] |
3. | Yamamoto K. Morphological studies of the spleen in idiopathic portal hypertension (so called Banti's syndrome without liver cirrhosis) using light microscopy, scanning electron microscopy and histometry. Acta Path Jap 1979;29:1-19. [PUBMED] |
4. | Chang CS, Li CY, Cha SS. Chronic idiopathic thrombocytopenic purpura. Splenic pathologic features and their correlation. Arch Pathol Lab Med 1993;117:981-5. |
5. | Wilkin's BS, Wright DH. Illustrated pathology of spleen. 1 st ed Cambridge university press; 2000. p. 144. |
6. | Klemperer P, Pollack AD, Baehr G. Pathology of disseminated lupus erythmatosus. Arch Pathol Lab Med 1941;32:569-631. |
7. | Gupta RK, Schuster R, Christian WD. A comparative immunohistochemical study of splenic arterial hyalinosis in health and disease. Am J Pathol 1972;69:79-88. [PUBMED] [FULLTEXT] |

Correspondence Address: Jyotsna Madan Sector 16 - A, House No - 1530, Vasundhra, Ghaziabad (U P) - 201 012 India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.55029

[Figure 1], [Figure 2] |
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