A condition known as thrombotic microangiopathy, characterized by the triad of thrombocytopenia; Low platelet levels, haemolytic anemia and damage to an organ due to the formation of thrombi at the capillary level showed the face of a serious complication not expected by a medical team at the Manatí Medical Center (MMC) and rare in the scientific literature .
Belinda Z Burgos
Latin Agency for Medicine and Public Health News
Thrombotic microangiopathy is a serious complication usually characterized by the triad of thrombocytopenia, which amounts to low platelet count, hemolytic anemia, which is a large-scale breakdown of red blood cells and damage to an organ due to the formation of thrombi at the capillary level. Epidemiology is still unclear due to poor identification.
And it’s that the clinical snowball that a Puerto Rican man manifested three years ago and at the age of 19 began with swelling in the legs from five months of evolution. At the time, he was on display at the external clinics of the residency program. On physical examination, vital signs were within normal parameters, but doctors noted swelling in both legs, a proteinuria, when the kidneys are working properly.
His treatment started at that time (2017) with a daily diuretic by mouth, but the patient did not return for follow-up appointments.
Three years later, he reported to the emergency room of the Manatí Medical Center (MMC) with the main complaint being headache, vomiting and generalized cramps from two weeks of evolution. On physical examination, she had elevated blood pressure, peripheral edema and edema in the face, pain on palpation in the epigastric region and edema in both legs, Dr. Liliana Colón Santos of the Department of Public Health continued in an interview with Medicine and Public Health (MSP General practice medicine of the said hospital institution.
“It was then decided to include the patient with uremic syndrome – serious clinical picture when the kidneys are severely damaged – and pancreatitis . It remains to be said that this was a long and complicated hospitalization that required the teamwork of several subspecialists. The main treatment consisted of hydration through a vein, antibiotics, blood pressure management and emergency dialysis, as well as a kidney biopsy. In the images we found edema in the pancreas, but the kidneys were of normal size “, Dr. Colón Santos explains.
“During hospitalization, our patient was complicated several times by hemolytic anemia – breakdown of red blood cells – requiring several blood transfusions. He also had several difficult-to-treat hypertensive crisis events. But after many successive drug and dialysis optimizations, we were able to stabilize the pressure and creatinine started to drop. “he added.
It also indicated the patient was negative for glomerulonephritis, rheumatologic and myeloproliferative disorders. However, the kidney biopsy reported 80% fibrosis and vascular thrombus. After a month of hospitalization, the patient was discharged with a diagnosis of permanent renal failure on dialysis and thrombotic microangiopathy, a challenging diagnosis in the literature.
Thrombotic microangiopathy is a serious complication usually characterized by the triad of thrombocytopenia, which consists of low platelet count, haemolytic anemia and damage to an organ due to the formation of thrombi at the capillary level. The epidemiology is still unclear due to its low identification or diagnosis.
Thrombotic microangiopathy had only two classifications. The first is purple thrombotic thrombocytopenia, which in most cases occurs with severe thrombocytopenia and neurological changes. The second is a shiga toxin-mediated hemolytic uremic syndrome – of bacterial origin – that presents with bloody diarrhea and is more common in children.
“Our case does not meet any of these criteria from those first two classifications. Now a third classification is emerging, known as complement-mediated hemolytic uremic syndrome. He is currently continuing with the patient, both in the dialysis unit and in the outpatient clinics ”, confirmed.
This condition is rare among the reported cases with an incidence of 7 per 1,000,000 population. For an accurate diagnosis of this syndrome it would be to be able to identify genetic mutations. Treatment consists of plasma exchange, treatment with eculizumab, an immunosuppressant, and may sometimes require a kidney transplant, the doctor explained.
“Our challenge as a GP now is to work with a young patient on permanent dialysis who is not 100 percent faithful to his treatment and does not fully understand what it means to be a kidney patient. The ideal for our patient would be an organ transplant due to permanent renal failure at a young age, but for that we need to keep working on both psychology and emotional state and eventually be able to do a genetic study It is our desire as health professionals to create awareness and educate our population to properly follow their medical appointments. Many conditions, if detected in time, with the patient’s teamwork and a good medical team, can stop their progression and sometimes even be cured“disclosed.
Doctors Edgar Hernández Montalvo, a nephrologist at Manatí Medical Center, Ignacio Acevedo Sierra and Beth Ann Rivera Fomenta, both family medicine practitioners, were part of this case report.