A 61-year-old man with a history of hypertension presented with asthenia, adynamia and fever of 38.5° axillary, two weeks of evolution, associated with the appearance of erythematous plaques with areas of inguinal peel.
The patient was admitted with stable vital signs, fever, skin lesions described, palpable hepatomegaly of three fingertips and enlarged spleen.
No lymphadenopathies.
A skin biopsy showed nonspecific urticarial pruritis.
At laboratory level white blood cell count was 3.OOOO per mm3 with polymorphonuclear predominance, hematocrit 29%, platelet count 95.OOOO mm3, LDH 9.5O GGT 125 mg/dl
Protein electrophoresis with hypoalbuminemia and mild alpha 2 increase.
Urinalysis was normal.
All viral and immunological serology were negative.
Four blood cultures and one urine culture were negative.
A computed axial tomography of the neck, thorax and abdomen showed hepatomegaly and rest without particularities.
PAMO showed hemophagocytosis.
As six of the criteria were met, the patient was diagnosed with HS and started therapy with high-dose corticoids and intravenous gammaglobulin at doses of 400 mg/kg/day for 5 days with fever disappearance.
On the days of administration of gamma globulin, the fever reappeared, with an increase in the degree of stenosis, so a diagnostic lymphoproliferative process was performed when malignancy was suspected.
The biopsy showed congestive stenosis, expansion of red pulp, extramedullary hematopoiesis, absence of neoplasia, signs of hemophagocytosis and liver wedge biopsy minimal steatosis sinus ectasia.
Therapy was initiated with eEtoposide 150 mg/m2.
The patient had severe tricythemia with high transfusion requirements and prolonged febrile neutropenia.
A new ABPM revealed medullary aplasia.
He developed multiple organ failure, consumption coagulopathy, massive bleeding and died 47 days after admission.
