CHAPTER 25 HISTIOCYTIC PROLIFERATIVE DISORDERS 387
lymphopenia and anemia are uncommon side effects; most common side effect is
vomiting
Azathioprine: 2 mg/kg or 50 mg/m2 PO q24h until remission; then EOD or twice
weekly.
Histiocytic sarcoma (malignant histiocytosis):
Typically nonresponsive
Rapidly progressive, and fatal
Fails to respond to therapy
Chemotherapy often trialed including corticosteroids, cyclophosphamide, vin-
cristine, and doxorubicin-based protocols
Fluid therapy or blood transfusions may be required depending on clinical
findings.
Malignant fibrous histiocytoma:
Surgical excision if possible
Poor prognosis due to local invasion and tendency to metastasize.
COMMENTS
Effectiveness of treatment is determined by repeated physical examinations, CBC and
biochemistry profiles, and diagnostic imaging.
Patients with systemic disorder have a fluctuating debilitating disease that can be
characterized by multiple clinical episodes and asymptomatic periods.
Prognosis for malignant disorder is extremely poor; death usually occurs within a few
months of diagnosis.
Fig. 25.1.Multiple cutaneous histiocytomas in a 2-year-old male-castrate Chinese shar pei. Additional nod-
ules were noted over most of the body.