What is sinus hyperplasia?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

Sinus hyperplasia

This disorder is a generally benign condition observed in lymph nodes, draining extremities, mesenteric regions, sites of malignant disorders, or foreign bodies. Erythrophagocytosis may be present in the involved lymph nodes. Sinuses are dilated and contain histiocytes. This is not a true histiocytic disorder but rather a normal lymph node response to draining antigen.

Sinus histiocytosis with massive lymphadenopathy (SHML)

Also called Rosai-Dorfman disease, [68, 69, 60] this is a usually persistent, massive enlargement of the nodes by proliferation and accumulation of histiocytes that are characterized by emperipolesis. [70] The disease is rarely familial. [70, 71, 72, 73]  SHML can occur after bone marrow transplant for acute lymphoblastic leukemia, after or concurrent with diagnosis of lymphoma, human herpesvirus 6 (HHV6), and EBV infections. [71, 72, 73]

Although the disease is rarely familial, [70] a rare familial variation, termed Faisalabad histiocytosis, has been described in 2 families. These individuals have multiple congenital abnormalities including fractures, short stature, hearing impairment, joint contractures, and massive enlarged lymph nodes resembling Rosai-Dorfman disease. The disorder appears to be transmitted as an autosomal recessive syndrome. [74]

SHML cells are positive for CD68, CD163, α-antichymotrypsin, α-antitrypsin, Fascin and HAM-56. SHML cells express moderate IL6 cytokine.

The male-to-female ratio is about 4:3, with a higher prevalence in blacks than in whites. Systemic symptoms, such as fever, weight loss, malaise, joint pain, and night sweats, may be present. Cervical lymph nodes are most characteristically involved, but other areas, including extranodal regions, can be affected. These disorders can manifest with only rash or bone involvement. [68, 70, 75]

Immunologic abnormalities can be observed, [76] including leukocytosis; mild normochromic, normocytic, or microcytic anemia; increased Ig levels; and abnormal rheumatoid factor. Positive results for lupus erythematosus have also been reported.

The disease is pathologically benign and has a high rate of spontaneous remission, but persistent cases requiring therapy have occurred. [70, 76, 77] In exceptional cases with obstructive complications, surgery, radiation therapy, and chemotherapy have been used to treat the disease. [70]  Death from SHML is known to occur. [76]


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