A 29-year-old black Haitian woman with a history of SCD, but only in Chile since 2017 due to language limitations.
It presents chronic damage already established for medical nephropathy with proteinuria in nephrotic range (4.6 g/day), old cerebral infarctions, severe cardiomegaly with preserved ejection fraction and infarcts with morphology in H.
Polytransfused during the diagnostic process, then hydroxyurea is prescribed and maintained with good outpatient follow-up.
In the last control, the patient presented hemoglobin (Hb) 5.5 g/dL, lactate dehydrogenase (LDH) 868 U/L, total bilirubin (BT) 2.78 mg/dL, with no RG incidents.
Five days later, the patient was committed to consciousness and mental illness, being discharged from the Complejo Asistencial Dr. Sótero del Río.
The patient was admitted with somnolence, poorly perfused, hypotensive (93/51 mmHg), tachypnea syndrome (109 bpm), fever (39°C), with hepatomegaly of O2 to 35%, superficial palpitations, 94%, other important.
Her admission tests showed Hb 3.3 g/dL, leukocytes 19.07 × 103/uL, BT 5.08 mg/dL, direct 2.54 mg/dL, LDH 1306 45 mg/L, reactive creatinine 1.31 mg/dL.
The clinical picture is interpreted as a hemolytic crisis of SCD and severe anemia, indicating a new transfusion of 4 units of RG, evolving with greater clinical deterioration, decrease of Hb to 2.8 g/dL requiring renal replacement therapy 119 mg mEq/dL
After ruling out the infectious origin and other complications of the SCD, the diagnosis of hypertonic syndrome and prednisone was raised by alloimmunization, with prescription of hydration with hypotonic serum (SF 0.45% and 0.45%).
A positive direct Coombs from the blood bank, as well as a cross-reaction to all available RG units, should be informed about transfusions.
Thus, after starting steroids, Hb levels progressively improved: 2.7 g/dL on day 3, 2.9 g/dL on day 5, 4.5 g/dL on day 12 and 5.8 g/dL on day 20.
At the same time, it recovers consciousness and normalizes renal function.
Due to suspicion of a RHTT, the Instituto de Salud Pública was requested to phenotyping erythrocytes, detecting the presence of anti-Fy3 antibodies directed against the Duffy system, suggesting transfusions in new black donors.
Finally, the patient developed stable Hb levels (range 5.0-5.5 g/dL) under steroidal therapy, hydroxyurea, vitamin B125.5 g/dL, biweekly erythropoietin, and high corticosteroid therapy.
