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CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 3  |  Page : 473-475
Nonhemophilic hemosiderotic synovitis of the knee: A case report and review of literature


Department of Pathology, Bhabha Atomic Research Center Hospital, Mumbai, Maharashtra, India

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Date of Web Publication14-Aug-2014
 

   Abstract 

Synovium is specialized mesenchymal tissue lining the inner surface of the joint capsule and is the site for a series of pathologic processes that are characteristic, and in some cases specific, to this distinctive tissue. Hemosiderotic synovitis is a rare and inadequately defined synovial proliferative disorder, which develops with recurrent hemorrhages in the joint. The most affected joint from bleeding is the knee whatever the etiology is. Repeated hemarthrosis may produce significant structural alteration of joints leading to chronic osteoarthritis. The most common cause is hereditary clotting factor deficiency diseases like hemophilia. We report a rare case of nonhemophilic hemosiderotic synovitis of the knee joint, in which the patient lacks history of any bleeding diathesis. Its definitive diagnosis was possible only by histopathological examination. The prompt recognition of this distinct subtype of hemosiderotic synovitis and awareness of underlying causes should lead to earlier diagnosis, appropriate therapy, less joint destruction, and better outcomes.

Keywords: Hemophilia, hemosiderotic, knee, nonhemophilic, synovitis

How to cite this article:
Jayalakshmi V, Chikhale NP, Mishra A, Cherian S. Nonhemophilic hemosiderotic synovitis of the knee: A case report and review of literature. Indian J Pathol Microbiol 2014;57:473-5

How to cite this URL:
Jayalakshmi V, Chikhale NP, Mishra A, Cherian S. Nonhemophilic hemosiderotic synovitis of the knee: A case report and review of literature. Indian J Pathol Microbiol [serial online] 2014 [cited 2020 May 29];57:473-5. Available from: http://www.ijpmonline.org/text.asp?2014/57/3/473/138779



   Introduction Top


Synovium is specialized mesenchymal tissue that is essential for the appropriate function of the locomotor apparatus. It is a glistening white membrane with delicate villous projections that lines the inner surface of the joint capsule. Synovium is the site for a series of pathologic processes that are characteristic, and in some cases specific, to this distinctive tissue. [1] Hemosiderotic synovitis is the distinctive type of proliferative synovial disorder develops as a result of chronic intra-articular hemorrhage. It follows chronic intra-articular hemorrhage, which can occur in patient with hemophilia and synovial hemangioma. The most affected joint from bleeding is the knee whatever the etiology is. [2] Correct etiological diagnosis needs a detailed analysis of both laboratory and radiodiagnostic tests. However, these tests may not always be helpful to diagnose some rare types such as hemosiderotic synovitis, which needs histopathological examination for definitive diagnosis. [3] In general, Patients with hemosiderotic synovitis complaint of pain and stiffness of the involved joint, because almost invariably, secondary osteoarthritis develops in association with chronic intra-articular hemorrhage. [1]

The knowledge and prompt recognition of this entity may lead to early identification of the underlying cause, which may help in appropriate therapy and prevention of complications. We report a rare case of nonhemophilic hemosiderotic synovitis of the knee in an elderly female along with a review of literature of this entity.


   Case report Top


A 73-year-old female patient with long standing osteoarthritis of bilateral knee joints, presented with increasing pain in the left knee joint along with swelling in the suprapatellar region. History and clinical investigations excluded a clotting factor deficiency and collagen vascular diseases. Furthermore, there was no history of anticoagulant drug intake. Prothrombin time, activated partial thromboplastin time, and platelet count were within the normal limits. Sickling test was negative with normal hemoglobin electrophoresis. A total knee replacement was done and excessive fat and synovium obtained from the suprapatellar pouch was sent for histopathological examination.

Grossly, the specimen consisted of a single, yellowish, soft, fibrofatty bit measuring 5.5 cm 4.0 cm 2.5 cm, with few rusty brownish areas.

Microscopically, the section showed marked proliferation of the synovium with a fine villous architecture [Figure 1]. The synovial lining cells contained deep brown hemosiderin pigment [Figure 2]. This was confirmed by Prussian blue staining [Figure 3]. Hemosiderin was also seen in the subsynovial connective tissue. There was no proliferation of mononuclear synovial cells, lipid-laden cells or multinucleated osteoclast like giant cells as seen in pigmented villonodular synovitis (PVNS). Hence, it was diagnosed as hemosiderotic synovitis.
Figure 1: Low power photomicrograph showing marked proliferation of the synovium with a fine villous architecture (H and E, ×100)

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Figure 2: High power photomicrograph showing fine granules of hemosiderin in the macrophages and surface synovial cells (H and E, 400)

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Figure 3: Photomicrograph demonstrating accumulation of iron localized in the macrophages and the synoviocytes on Prussian blue staining confirming deposition of hemosiderin (Prussian blue stain, ×100)

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   Discussion Top


Synovial pathologies may present as acute or chronic synovitis. Chronic synovitis refers to a persistent, nonspecific, proliferative lesion of the synovium, usually monoarticular, with little or no involvement of the articular cartilage or bone, and without clear evidence for any other primary pathological process. [3] Hemosiderotic synovitis develops with recurrent intra-articular hemorrhages. The most common cause is hereditary clotting factor deficiency diseases such as hemophilia. The most commonly involved joint is the knee. Causes of hemosiderotic synovitis other than hemophilia include oral anticoagulant use, trauma, rheumatoid or psoriatic arthritis, osteoarthritis, collagen vascular diseases, PVNS, hemochromatosis, scurvy, sickle cell anemia, synovial hemangioma and myeloproliferative diseases. [4]

The gross appearance of the synovium is almost similar in hemosiderotic synovitis and PVNS. Both appear brown or rust colored with the loss of normal glistening translucency of the synovial membrane. With repeated hemorrhage, the synovium becomes thickened and opaque owing to intrasynovial and subsynovial fibrous scarring. Clinical and radiological findings may not yield a definitive diagnosis, which may only be possible by histopathological examination, as in this case. [5]

In hemosiderotic synovitis, the hemosiderin is confined mainly to the synovial lining cells. Hemosiderotic synovitis generally has a much finer villous appearance in contrast to the plump papillary pattern seen in PVNS. It lacks the distinct submembranous mononuclear and giant cell proliferation with hemosiderin deposition that characterizes PVNS. [6]

A hyperplastic, vascular tissue is formed in the synovial membrane within few days of bleeding. With the breakdown of hemoglobin, iron containing hemosiderin is released and stored. This accumulation leads to rusty brown discoloration of the tissue. More bleeding episodes result in further deposition of hemosiderin and darker discoloration. [4]

The results from the studies show that the iron deposits at localized sites in the synovium are associated with the production of pro-inflammatory cytokines and an ability to inhibit the formation of human cartilage matrix. This supports the hypothesis that iron plays a leading role in the induction of synovial changes and the consequent production of catabolic mediators that are harmful to articular cartilage. It is not clear whether hemosiderin is directly involved in the stimulation of cytokine production; it seems more likely that phagocytosis by synovial cells and blood macrophages released into the hemarthritic joint leads to the stimulation of cytokine production. [7]

Yalçin et al. [3] described the similar case in 20-year-old female patient and concluded that when more frequent etiologies are excluded in recurrent joint effusions, hemosiderotic synovitis should be considered among rare causes. Differential diagnosis with PVNS should be made with histopathological examination.

Krebs [8] stated that patients with hemosiderotic synovitis might benefit from arthroscopic intervention as patients with PVNS and that arthroscopy and improvements in clinical characterization led to a better understanding of the association between synovial abnormalities and degeneration.

Hemosiderotic synovitis is often associated with severe degenerative articular cartilage disease. Hence, the treatment revolves around surgical and conservative management of the arthritis. [2],[4]

A subset of hemosiderotic synovitis is caused by intrasynovial hemangiomas. Fewer than 200 cases have been reported. Knee joint is the most frequently affected joint. The hemorrhage is rarely as progressive as in bleeding diathesis and accelerated osteoarthritis is usually not a feature. [9]


   Conclusion Top


Nonhemophilic hemosiderotic synovitis is a type of hemosiderotic synovitis occurring in patients without history of bleeding diathesis. Definitive diagnosis is usually not possible by clinical and radiological methods. Hence, histopathological examination is mandatory. Its prompt recognition and awareness of underlying causes should lead to earlier diagnosis, appropriate therapy, less joint destruction, and better outcomes. Research shows that the intra-articular blood (mainly iron) stimulates cytokine release, which lead to inflammation and joint destruction. Further studies are needed to confirm the exact component of blood and definitive mechanism leading to the changes of hemosiderotic synovitis.

 
   References Top

1.
O'Connell JX. Pathology of the synovium. Am J Clin Pathol 2000;114:773-84.  Back to cited text no. 1
    
2.
Humphrey PA. Joints and synovium. In: Humphrey PA, Dehner LP, Pfeifer JD, editors. The Washington Manual of Surgical Pathology. 2 nd ed. PA: LWW; 2012. p. 822-5.  Back to cited text no. 2
    
3.
Yalçin N, Bektaþer B, Ciçekli O, Uðraþ S, Doðan M. An unusual cause of recurrent joint effusions: Nonhemophilic hemosiderotic synovitis of the knee. Acta Orthop Traumatol Turc 2010;44:162-5.  Back to cited text no. 3
    
4.
France MP, Gupta SK. Nonhemophilic hemosiderotic synovitis of the shoulder. A case report. Clin Orthop Relat Res 1991;262:132-6.  Back to cited text no. 4
    
5.
Roosendaal G, Lafeber FP. Joint damage as a result of hemarthrosis. In: Caviglia HA, Solimeno LP, editors. Orthopedic Surgery in Patients with Hemophilia. 1 st ed. Italy: Springer; 2008. p. 5-14.  Back to cited text no. 5
    
6.
Bullough PG. Joint diseases. In: Mills SE, editor. Sternberg's Diagnostic Surgical Pathology. 5 th ed. PA: LWW; 2010. p. 211-2.  Back to cited text no. 6
    
7.
Roosendaal G, Vianen ME, Wenting MJ, van Rinsum AC, van den Berg HM, Lafeber FP, et al. Iron deposits and catabolic properties of synovial tissue from patients with haemophilia. J Bone Joint Surg Br 1998;80:540-5.  Back to cited text no. 7
    
8.
Krebs VE. The role of hip arthroscopy in the treatment of synovial disorders and loose bodies. Clin Orthop Relat Res 2003;406:48-59.  Back to cited text no. 8
    
9.
Devaney K, Vinh TN, Sweet DE. Synovial hemangioma: A report of 20 cases with differential diagnostic considerations. Hum Pathol 1993;24:737-45.  Back to cited text no. 9
    

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Correspondence Address:
Nitin P Chikhale
Department of Pathology, Bhabha Atomic Research Center Hospital, Anushaktinagar, Mumbai - 400 094, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.138779

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    Figures

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