A 28-year-old man with an Alport syndrome who after 5 years on hemodialysis received a kidney transplant (KTx) from a deceased donor.
On the fourth year of hemodialysis, the patient presented pan-cites.
Myelogram showed dysmorphic features suggestive of myelodysplasia, and the karyotype in bone marrow was normal.
An autoimmune etiology was presumed and cyclosporine (CsA) was indicated.
At one month, leukocytes had risen from 2,700 to 4,000 mm3, platelets from 126,000 to 160,000 mm3, and hematocrit from 27 to 36.5%.
She continued receiving CsA until the KTx, 15 months later.
Prior to transplantation, because it would not be necessary to use azathioprine because of its predatory effect, honey was prescribed on its daily use of mycophenolate mofetil (MMF) in progressive doses up to 2,000.
Epidermitis was not caused during 6 weeks of usage; however, it could be used in a future RTx.
On the day of KTx, CsA, MMF and corticosteroids were prescribed.
Although the graft produced urine immediately, the clearance was low, so it was dialyzed on 3 occasions.
On day 8 a right iliofemoral thrombophlebitis was diagnosed, starting anticoagulant therapy.
On day 12 she had diarrhea, vomiting and colic pain, which led to a reduction in the dose of MMF and to prescribe metronidazole and ciprofloxacin, due to the possibility of an infectious etiology.
Coprocultives, coproparasitological, Clostridium dijficile toxin and rotavirus were negative.
The diarrhea subsided completely 7 days later.
On day 26 she presented headache and a new episode of diarrhea, vomiting and abdominal pain.
She had no fever.
The scanner showed mild stenosis, thickening of the intestinal wall, pleural effusion, abundant ascites and lymphocele on the anterior side of the laugh, which shifted towards dorsal.
Laboratory tests showed elevated C-reactive protein (CRP) to 18.7 mg/L (VN: < 1) and severe hypoalbuminemia (1.8 g/d).
Metronidazole and ceftriaxone were prescribed.
No infection was demonstrated and diarrhea subsided in 5 days.
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On day 32 she suddenly presented severe pain in the graft area with elevated creatine and uremia, metabolic acidosis and reduced urinary volume.
A scanner (requested for suspected renal rupture) showed ascites of greater magnitude, thickening of the colon wall and increased size of the lymphocele, maintaining changes in the anterior scanner.
No rupture was observed.
Acute rejection was diagnosed and treated with 1 gram methylprednisolone for 3 days, replacing CsA by tacrolimus.
Azotemia decreased until creatinine stabilized at 2.4 mg/dl. Lymphocele was punctured obtaining 725 mi.
On day 38 there was fever > 38°c without apparent cause, associated with a rapid fall in hematocrit (34.5 to 23.8%), leukopenia (3,000 mm3), thrombocytopenia (130,000 mm3) and elevated CRP.
A direct Coombs test was negative.
MMF was suspended due to suspicion of spinal cord depression.
In the following days, the patient continued to develop anemia (To 20.7%), leukopenia (2,700 mm3) was accentuated, hypoalbuminemia persisted (1.8 g/dl), panhypoglobulinemia appeared.
Lymphocele began to deform the abdominal wall and marsupialization was performed on day 43.
The persistence of anemia and leucopenia led to post-transplant lymphoproliferative disease (PTLD) or recurrence of myelodysplasia.
Myelogram and flow cytometry ruled out PTLD.
Bone marrow showed abundant histiocytes and hemophagocytosis.
With a presumptive diagnosis of FAS scheduled for transplantation, MMF was suspended and tacrolimus was changed to CsA, maintaining prednisone at 10 mg/day.
Ferritin and triglyceride concentrations were high (1,873 ng/ml and 228 mg/dl, respectively) and normal fibrinogen (360 mg/dl).
CRP was normalized 24 h after the start of CsA and leukocyte count at 72 h.
In the following days the general condition improved progressively, the fever subsided and the hematocrit increased slowly but the renal function remained stationary (creatinine 2.6 mg/dl).
Renal biopsy showed acute rejection BANFFII b, arteriole and proliferative glomerular lesion, which was attributed to the donor.
Antinuclear antibodies, ANCA and C'3 and C'4 concentrations were normal.
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At day 56, creatininemia was 2.7 mg/dl and there were no signs of inflammation and quantification of IgGJgA and IgM immunoglobulins and albuminemia were normal.
The patient was discharged.
At the four-week follow-up visit, the outpatient clinic parameters had normalized and renal function was better (creatinine 2.4 mg/dl, creatinine clearance 33 ml/min/1.73 m2).
