ROSAI-DORFMAN (1969)DISEASE (EXTRANODAL SINUS HISTIOCYTOSIS) WITH MASSIVE LYMPHADENOPATHY (SHML)

ABOUT 500 CASES—-RARE

IDIOPATHIC—SELF LIMITED IN MOST CASES ?INFECTIOUS ETIOLOGY?

MASSIVE B, NON-TENDER, PAINLESS CERVICAL LAD

EXTRANODAL IN 40% OF CASES

MOST OFTEN INVOLVES THE H&N

LOW GRADE FEVER

LEUKOCYTOSIS (NEUTROPHILIA)AND POLYCLONAL HYKPERGAMMAGLOBULINEMIA—ENGULFEMENT OF (EMPERIOPOSESIS) OF LYMPHOCYTES AND S-100 POSITIVITY

POLYMORPHOUS CELLULAR INFILTRATE—LYMPHOCYTES, PLASMA CELLS, AND HISTIOCYTES (SHEETS OR CLUSTERS WITH RUSSEL BODIES)

ANEMIA

HIGHER SED RATE

BLACK>WHITE

FEMALE>MALE

Rx: SUPPORTIVE AND SYMPTOMATIC–STERIODS, ABX, RT, CHEMO AND SURGERY REPORT VARIABLE RESULTS