Case
Our patient is a 42 ‐year‐old
female with a history of end stage kidney disease due to hypertension,
she is 18 months post deceased donor
kidney transplant. Her maintenance immunosuppression included
mycophenolic acid 360 mg, 4 times a day,
prednisone 10 mg daily and monthly belatacept infusion. she had an episode of
antibody mediated rejection for which she received IV methylprednisone, plasma
exchange, high dose IVIG with improvement of allograft function 1 year post transplant.
Patient presented to the emergency room
with abdominal pain, chills, and dysuria of 1 week
duration. Vital signs were within normal
limits. On examination, she had mild epigastric tenderness and
splenomegaly. Laboratory studies were significant for leucopenia
with, WBC of 2000 k/uL, elevated liver enzymes
including AST 97 U/L, ALT 150 U/L, ALP 302 iu/l, ferritin
of 11792 ng/ml and triglyceride
449 mg/dL.
CT scan of the abdomen and pelvis without contrast showed
splenomegaly and hepatic steatosis. Viral hepatitis studies were negative.
Based on the clinical presentation,
HLH was suspected. CT chest without contrast showed tree-in-bud opacities in the right lower lobe and
Scattered pulmonary nodules measuring up to 0.6 cm.
Bone marrow biopsy was done on day 4 that showed cellular marrow with
active trilineage hematopoiesis with no evidence of a hemophagocytosis and no
evidence of histoplasmosis or other infections. At this time,
patient was empirically started on dexamethasone 40 mg
daily and posaconazole 300 mg orally twice a day.
Soluble serum IL-2R, was
4806 pg/ml and CXCL9 was 7,244 pg/ml,.
both of which markedly elevated (>20 ng/ml),that
is consistent with the diagnosis of HLH.
On day 7 of admission, urine histoplasma antigen
returned at a level above the limit of quantification, consistent with
disseminated histoplasmosis as the culprit
agent precipitating her HLH syndrome. At this point,
patient was treated with IV liposomal amphotericin 3 mg/kg for 7 days then
switched to itraconazole 2.5mg/kg orally three times daily for 3 days as
loading dose, then transitioned to itraconazole
200mg orally twice a day for 12 months. Immunosuppressive regimen was changed
to cyclosporine and prednisone. Belatacept and mycophenolic acid were stopped.
Repeat urine histoplasma antigen was down to 8.35 ng/ml after 1 month. Three months later, patient
was asymptomatic, had normal WBC, platelets count, creatinine, AST, ATL, stable
hemoglobin of 8.4 g/dL, ferritin was down to 2454 ng/ml. Urine histoplasma
antigen was down to 6.6 ng/ml. patient had recovery of splenomegaly.