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February 1995, Vol 149, No. 2 >
Article | February 1995

Severe Thrombocytopenia as a Presenting Manifestation of Kawasaki Disease FREE

John S. Venglarcik, MD; Mouhab Ayas, MD; Tim Woods, MD
Arch Pediatr Adolesc Med. 1995;149(2):215-217. doi:10.1001/archpedi.1995.02170140097019.
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Kawasaki disease (KD) is an acute febrile illness of childhood that is characterized by conjunctival hyperemia, inflammation of the oral mucosa, characteristic changes in the extremities, cutaneous manifestations, and cervical adenopathy. The disease is most commonly seen in children younger than 5 years of age and is distinctly uncommon after adolescence.1

There is no diagnostic laboratory test for KD. However, elevated platelet counts are a consistent feature of this particular illness.2 Thrombocytosis was believed to be so central to the diagnosis that the presence of thrombocytopenia suggested an alternate diagnosis.3,4

Thrombocytopenia has been reported in KD.5-10 Twelve patients5-8 have had thrombocytopenia that was unassociated with idiopathic thrombocytopenic purpura. However, none of these individuals had a decrease in platelet count to a level that has been associated with spontaneous hemorrhage.11 Additionally, the thrombocytopenia was not the presenting manifestation. Three patients have been described with KD

REFERENCES

1 +
Butler DF, Hough DR, Friedman SJ, Davis HE.  Adult Kawasaki syndrome . Arch Dermatol . 1987;;123:1356-1361.
2 +
Morens DM, Melish ME.  Kawasaki disease . In: Feigin RD, Cherry JD, eds. Textbook of Pediatric Infectious Disease . 3rd ed. Philadelphia, Pa: WB Saunders Co; 1992;:2123-2139.
3 +
Yanagihara R, Todd JK.  Acute febrile mucocutaneous lymph node syndrome . AJDC . 1980;;134:603-614.
4 +
Melish ME.  Kawasaki syndrome . Pediatr Rev . 1980;;2:107-114.
5 +
Hara T, Mizuno Y, Akeda H, et al.  Thrombocytopenia: a complication of Kawasaki disease . Eur J Pediatr . 1988;;147:51-53.
6 +
Krowchuk DP, Kumar ML, Vielhaber MM, Danish EH.  Kawasaki disease presenting with thrombocytopenia . AJDC . 1990;;144:19-20.
7 +
Tatara K, Fujimaki W, Baba K, et al.  Diagnostic problems of atypical Kawasaki disease [in Japanese] . Prog Med . 1986;;6:150-154.
8 +
Yoshida S, Kaku S, Furusho K, et al.  An infant of mucocutaneous lymph node syndrome who had thrombocytopenia at the early stage developed bilateral coronary and axillary aneurysms [in Japanese] . Acta Paediatr Jpn . 1976;;80:389-396.
9 +
Shimizu T, Tochimaru H, Satake Y, Sotooka T.  A case of mucocutaneous lymph node syndrome associated with idiopathic thrombocytopenic purpura [in Japanese] . Shonika Rinsho . 1980;;33:1458-1462.
10 +
Lipnick RN, Lupan NLC.  Kawasaki disease with thrombocytosis followed by thrombocytopenic purpura in the same patient . AJDC . 1989;;143:139-140.
11 +
Shuman M.  Hemorrhagic disorders: abnormalities of platelet and vascular function . In: Wyngaarden JB, Smith LN, Bennett JC, eds. Cecil Textbook of Medicine . 19th ed. Philadelphia, Pa: WB Saunders Co; 1992;:987-999.
12 +
Barron K, DeCunto C, MontalvoJ, Orson F, Lewis D.  Abnormalities of immunoregulation in Kawasaki syndrome . J Rheumatol . 1988;;15:1243-1249.
13 +
Ono S, Onimaru T, Kawakami K, Hokonohara M, Miyata K.  Impaired granulocyte chemotaxis and increased circulating immune complexes in Kawasaki disease . J Pediatr . 1985;;106:567-570.
14 +
Barron K, Montalvo J, Joseph AK, et al.  Soluble interleukin-2 receptors in children with Kawasaki syndrome . Arthritis Rheum . 1990;;33:1371-1377.
15 +
Leung DY, Burns JC, Newburger JW, Geha RS.  Reversal of lymphocyte activation in vivo in the Kawasaki syndrome by intravenous gammaglobulin . J Clin Invest . 1987;;79:468-472.
16 +
Kawada K, Terasaki PI.  Evidence for immunosuppression by high-dose gammaglobulin . Exp Hematol . 1987;;15:133-136.
17 +
Burns JC, Glode MP, Clarke SH, Wiggins J, Hathaway WE.  Coagulopathy and platelet activation in Kawasaki syndrome: identification of patients at high risk for development of coronary artery aneurysms . J Pediatr . 1984;;105:206-211.

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References

1 +
Butler DF, Hough DR, Friedman SJ, Davis HE.  Adult Kawasaki syndrome . Arch Dermatol . 1987;;123:1356-1361.
2 +
Morens DM, Melish ME.  Kawasaki disease . In: Feigin RD, Cherry JD, eds. Textbook of Pediatric Infectious Disease . 3rd ed. Philadelphia, Pa: WB Saunders Co; 1992;:2123-2139.
3 +
Yanagihara R, Todd JK.  Acute febrile mucocutaneous lymph node syndrome . AJDC . 1980;;134:603-614.
4 +
Melish ME.  Kawasaki syndrome . Pediatr Rev . 1980;;2:107-114.
5 +
Hara T, Mizuno Y, Akeda H, et al.  Thrombocytopenia: a complication of Kawasaki disease . Eur J Pediatr . 1988;;147:51-53.
6 +
Krowchuk DP, Kumar ML, Vielhaber MM, Danish EH.  Kawasaki disease presenting with thrombocytopenia . AJDC . 1990;;144:19-20.
7 +
Tatara K, Fujimaki W, Baba K, et al.  Diagnostic problems of atypical Kawasaki disease [in Japanese] . Prog Med . 1986;;6:150-154.
8 +
Yoshida S, Kaku S, Furusho K, et al.  An infant of mucocutaneous lymph node syndrome who had thrombocytopenia at the early stage developed bilateral coronary and axillary aneurysms [in Japanese] . Acta Paediatr Jpn . 1976;;80:389-396.
9 +
Shimizu T, Tochimaru H, Satake Y, Sotooka T.  A case of mucocutaneous lymph node syndrome associated with idiopathic thrombocytopenic purpura [in Japanese] . Shonika Rinsho . 1980;;33:1458-1462.
10 +
Lipnick RN, Lupan NLC.  Kawasaki disease with thrombocytosis followed by thrombocytopenic purpura in the same patient . AJDC . 1989;;143:139-140.
11 +
Shuman M.  Hemorrhagic disorders: abnormalities of platelet and vascular function . In: Wyngaarden JB, Smith LN, Bennett JC, eds. Cecil Textbook of Medicine . 19th ed. Philadelphia, Pa: WB Saunders Co; 1992;:987-999.
12 +
Barron K, DeCunto C, MontalvoJ, Orson F, Lewis D.  Abnormalities of immunoregulation in Kawasaki syndrome . J Rheumatol . 1988;;15:1243-1249.
13 +
Ono S, Onimaru T, Kawakami K, Hokonohara M, Miyata K.  Impaired granulocyte chemotaxis and increased circulating immune complexes in Kawasaki disease . J Pediatr . 1985;;106:567-570.
14 +
Barron K, Montalvo J, Joseph AK, et al.  Soluble interleukin-2 receptors in children with Kawasaki syndrome . Arthritis Rheum . 1990;;33:1371-1377.
15 +
Leung DY, Burns JC, Newburger JW, Geha RS.  Reversal of lymphocyte activation in vivo in the Kawasaki syndrome by intravenous gammaglobulin . J Clin Invest . 1987;;79:468-472.
16 +
Kawada K, Terasaki PI.  Evidence for immunosuppression by high-dose gammaglobulin . Exp Hematol . 1987;;15:133-136.
17 +
Burns JC, Glode MP, Clarke SH, Wiggins J, Hathaway WE.  Coagulopathy and platelet activation in Kawasaki syndrome: identification of patients at high risk for development of coronary artery aneurysms . J Pediatr . 1984;;105:206-211.

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