This is a 37-year-old male patient with no personal history of interest, except for recurrent severe arthritis, ex-smoker and sporadic cannabis use, who came to the emergency room for odynophagia.
On physical examination there was nothing remarkable except mild overweight (body mass index: 29 kg/m2), with hyperemic tonsils.
Given the poor general condition of the patient, the presence of tonsils that prevent febricula (37.3oC) and the presence of tonsils, it was decided to enter Otorhinolaryngology on April 25, 2013.
On the day following admission, the patient had normal plasma platelets due to granulocytosis in charge of the haematology department under study, following laboratory tests showing mild anaemia (haemoglobin [Hb]: 11.9 g/dl; borderline white blood cell); leukopenia
Pharyngeal cultures and serology of cytomegalo, Epstein- rubeola, toxoplama, human immunodeficiency virus and herpes are negative.
The day after the transfer, a control laboratory was performed showing 8290 leukocytes, Hb 11 g/dl, platelets 20000/l, urea 59 mg/dl and Crp 2.3 mg/dl. A new pathological smear of peripheral blood was performed.
Some of them with ice cream core, erythroblastosis with atypical promyelocytes.
Compatible with acute myeloid leukemia probably promyelocytic, a myelogram was subsequently performed to confirm the promyelocytic cell infiltrate with a cytogenetic study confirming the translocation (t15;17).
That same day, we are consulted because the patient presented at night a sudden abdominal pain, focused on the lumbar level, with oligoanuria; the smear of peripheral blood around 8% is detected.
This context makes it very possible to diagnose thrombotic microangiopathy.
On April 27, 2013, an abdominal computerized axial tomography (CAT) showed perfusion defect at the level of the bilateral renal cortex, with permeable renal artery and vein, compatible with cortical necrosis.
Renal scintigraphy is also performed, which is indicative of cortical necrosis.
Blood tests performed 6 h later showed sediment with microhaematuria and proteinuria of 100 mg/dl, laboratory test with normal serum creatinine levels of 4.6 mg/dl, blood coagulation with mild elevation of hepatic enzymes with normal C-reactive protein
The next day the patient presented with oliguria with a Crp of 7 mg/dl and high blood pressure (160/80 mmHg), so hemodialysis was initiated (first session: April 28, 2013).
A total of 12 sessions of plasmapheresis (7 consecutive and 5 alternate days) are performed until haemolysis data disappear (decrease in lactate dehydroglobine [LDH], 75 mg indirect bilirubin [17dl]), May 2013
On May 1, 2013, at the end of the fifth session of plasmapheresis, the patient presents with severe respiratory failure together with mild hemoptysis and decreased level of consciousness, so he goes to the intensive care unit (ICU).
Mechanical ventilation is required in the ICU and lung scintigraphy is performed for suspected pulmonary infarctions that do not show evidence of pulmonary thromboembolism; a cranial CAT scan is also performed to rule out cerebral infarctions.
Finally, the patient leaves the ICU four days later with spontaneous breathing with respiratory infection by Acinetobacter and Pseudomona aeruginosa and urinary infection by Acinetobacter baumanii.
The clinical response to chemotherapy with ATRA plus idarurrubicin is satisfactory and has a good response to treatment, being given by high-risk patients on June 18, 2013 with outpatient visits.
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From the renal point of view, the patient has remained anuric since then, requiring hemodialysis, with no signs of recovery.
Control CT angiography showed cortical hypoperfusion.
Once the platelets were normalized with chemotherapy treatment, percutaneous renal biopsy was performed (7 June 2013): with 19 glomeruli, 16 of which presented severe changes characteristic of coagulation necrosis secondary to complete destruction of ischemia.
In three of them the same is observed, but with preservation of their nuclei, as well as angiogram, thickening of membranes with presence of fibrin or arterioles, necrosis semi-clear (cystocytes), occasional polymorphous necrosis (PM).
In the interstitial area, diffuse necrosis is evident, with presence in the area of the corticomedullary junction and tubules with cylinders marked inflammatory infiltrates, fibrosis of the cortical atypia, chronic cellular ischemic patches
At the vascular level, medium caliber arteries, presence of intimal mucoid degeneration with luminal occlusion and incipient myointimal fibrous changes, which confirms the diagnosis of coagulative cortical necrosis with HUS in patients with vascular changes.
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Diagnosis
Acute renal failure due to bilateral cortical necrosis of ischemic cause by thrombotic microangiopathy in the context of myeloid crisis of acute myeloid leukemia.
