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September 12 th , 2011. Good Morning. Incomplete (atypical) Kawasaki. Incomplete Kawasaki. Infants are more likely to present with incomplete KD, even presenting only with fever and no other clinical features

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  1. September 12th, 2011 Good Morning

  2. Incomplete (atypical) Kawasaki

  3. Incomplete Kawasaki • Infants are more likely to present with incomplete KD, even presenting only with fever and no other clinical features • Infants are also at increased risk of CA aneurysms, because of delay in treatment due to their lack of complete diagnostic criteria • Thus, infants six months of age or less with unexplained fever ≥ 7 days should be evaluated for KD regardless of whether they have signs of mucocutaneousinflammation

  4. Incomplete Kawasaki • Continued consideration of alternative diagnoses in “atypical cases” is essential despite initial response to IVIG • This is particularly important in children who fail to respond or respond only incompletely to IVIG

  5. HLH • Hemophagocyticlymphohistiocytosis • Proliferation of cells of the mononuclear phagocyte system • Familial – underlying genetic disorder • Secondary- caused by acquired conditions, including viral, neoplastic, and rheumatologic diseases

  6. Familial HLH • Autosomal recessive • Mutation in the perforin gene accounts for 20-40% • Incidence 1.2 per million • Presents in infancy (birth to 18 months) • Negative family history does not exclude • Hyperactiviation of T cells and macrophages = cytokine storm • TNF-alpha, IL1, IL6, and interferon gamma

  7. Clinical Features • Prolonged fever and hepatosplenomegaly • LAD, rash, and jaundice • May mimic viral infections, leukemia, multiorgan failure due to sepsis, and encephalitis • CNS involvement • Develops later in course • Nonspecific • Irritability, neck stiffness, hypo- or hypertonia, convulsions, ↑ICP, 6th or 7th cranial nerve palsy, atxia, bulging fontanelle

  8. Lab abnormalities • The pathologic findings result from the aggressive proliferation of normal histiocytes and T-lymphocytes in various tissues • Cytopenias, hypertriglyceridemia, coagulopathy, elevated ferritin and transaminases • CSF typically shows hyperproteinemia and pleocytosis

  9. Bone Marrow Biopsy Reactive histiocytes show phagocytosis of nucleated red blood cells (red arrows) and platelets (black arrows). *In nearly 20 percent of cases, documenting hemophagocytosis on the first bone marrow specimen is difficult or impossible

  10. Histiocyte Society *5 of 8 criteria must be fullfilled to diagnose *Unless there is a gene mutation

  11. Treatment • Treat any underlying conditions • Chemotherapy • Etoposide, corticosteroids, cyclosporine, and IT MTX • Stem cell transplant • Best overall cure rates

  12. Prognosis • Familial HLH is fatal • Median survival is less than 2 months if untreated

  13. Take Home Message • If an infant presents with fever, hepatosplenomegaly, and cytopenia, HLH should be high in the differential • Early diagnosis of HLH is crucial because prognosis depends on prompt treatment

  14. Noon Conference Jaundice/Anemia in the Newborn, Dr. Wetzel

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