A 41-year-old woman with a history of obesity and mild cutaneous and joint systemic lupus erythematosus presented with fever without other discomfort for 48 h.
As a relevant history, the patient had undergone a sleeve gastrectomy 3 weeks before the onset of symptoms.
On admission, fever and physical examination showed no obvious changes, with surgical wounds without signs of infection.
Laboratory tests showed a complete blood count without anemia, with a mean corpuscular volume in 78 FML, slightly elevated CRP levels in 78 urodynamically sensitive transaminases (ULOT 60 U/L faecalis, and elevated total bilirubin 31 U/L).
Lupic activity tests were negative.
Given recent bariatric surgery and low MCV, ferritin was requested that was high (1,302 IU/L) and interpreted as secondary to acute infection.
Antibiotic treatment was started achieving transient improvement, but persistent fever.
An abdominal CAT scan ruled out gastrectomy and showed stenosis and retroperitoneal adenopathies in splenic hilium and iliac chains.
Control tests showed progressive hypochromic microcytic anemia, normal, urocultive and hemocultive CRP without bacterial growth, respiratory molecular viral panel and lupus activity tests both negative.
A CT scan of the chest, abdomen and abdomen showed mild bilateral pleural effusion, nonspecific pulmonary nodules, multiple supra- and supra-fragency anomalies smaller than 1 cm and persistent infradian pelvis was requested.
A non-invasive study of the pulmonary nodules was carried out, which resulted negative (beta-glucan, mannitol, CMV PCR, HIV, IgM chlamydia and PCR pneumocystis jiroveci).
She was diagnosed with lymphoproliferative syndrome and PET CT was requested, which showed an increase in the glucose uptake of lymph nodes and spleen (SUV greater than 8).
Lymph node biopsy could not be performed due to difficult surgical access.
The patient showed progressive increase of LDH, transaminases and triglycerides and developed pancreatitis.
A new ferritin was requested, which resulted in 6,482 IU/L, and a possible HPS was proposed.
A bone marrow study showed increased cellularity without evidence of hemophagocytosis and without malignancy.
Liver tests and LDH tests were negative to perform biopsy (LDH 3.585 U/L, GOT 1063 U/L, GPT 320 U/L, GGT 674 U/L, FA 330 mg%).
Times after liver biopsy the patient developed extensive hepatic hematoma.
Bleeding was controlled by selective embolization by interventional radiology.
During evolution, an increase in ferritin to 74,583 U/L and LDH to 4,124 U/L was observed.
After the beginning of the CMT protocol HLH 04 liver tests returned to normal and ferritin decreased to normal.
A sister of the patient was studied for the possibility of performing consolidation allo-HSCT, resulting HLA identical.
At the time of writing this report, after 1 and 1⁄2 years of follow-up, the patient has good quality of life without need for transplantation.
