Thrombotic microangiopathy: symptoms

Symptoms of thrombotic microangiopathy are diverse. They can be divided into three groups:

Symptoms of a typical hemolytic-uremic syndrome

A typical post-diarrheal hemolytic-uremic syndrome is preceded by a prodrome, manifested in the majority of patients with bloody diarrhea lasting from 1 to 14 days (an average of 7 days). By the time of admission to hospital, 50% of patients have already stopped diarrhea. Most children have vomiting, mild fever, intense abdominal pain, imitating the picture of "acute abdomen." Following a diarrheal prodrome, an asymptomatic period of different duration may occur.

Symptoms of hemolytic-uremic syndrome are the following: sharp pallor, weakness, inhibition, oligoanuria, although diuresis does not change in some cases. Possible development of jaundice or dermal purpura.

Most patients develop oliguric acute renal failure, in 50% of cases requiring treatment with glomerulonephritis. However, observations with little or no renal dysfunction have been described. The duration of anuria on average is 7-10 days, the long duration is prognostically unfavorable. Arterial hypertension develops in most patients, usually mild or moderate, disappearing by the time of discharge from the hospital. The urinary syndrome is represented by proteinuria not exceeding 1-2 g / day and microhematuria. Possible the development of macrohematuria and massive proteinuria with the formation of nephrotic syndrome. In most cases, due to hemolysis of erythrocytes, pronounced hyperuricemia is observed, not proportional to an increase in the levels of creatinine and urea. By the time of admission to hospital, patients can either be dehydrated due to intestinal fluid loss, or, more often, hyperhydrated due to anuria.

Hyperhydration and hypertension in combination with severe anemia and uremia can be complicated by congestive circulatory insufficiency.

CNS lesion is noted in 50% of children with post-diarrheal hemolytic-uremic syndrome and can manifest as seizures, drowsiness, visual impairment, aphasia, confusion, coma. In 3-5% of cases, the development of cerebral edema is possible. The cause of neurological disorders may be hyperhydration and hyponatremia, which have developed as a result of diarrhea. However, it is now believed that these factors only aggravate the microangiopathic lesion of the brain.

Lesions of the gastrointestinal tract in patients with hemolytic-uremic syndrome develops as a result of severe microcirculatory disorders and may manifest as a heart attack or intestinal perforation, intestinal obstruction. In some cases, severe hemorrhagic colitis can be complicated by prolapse of the rectum, which is a prognostically unfavorable factor. In 30-40% of patients, hepatosplenomegaly is noted, in 20% - pancreatic disease with the development of diabetes mellitus, and in the most severe cases - organ infarction.

Rare symptoms of hemolytic-uremic syndrome - the defeat of the lungs, the eyes, the development of rhabdomyolysis.

Symptoms of atypical hemolytic-uremic syndrome

Atypical hemolytic-uremic syndrome often develops in adolescents and adults. The disease is often preceded by a prodrome in the form of a respiratory viral infection. Sometimes symptoms of gastrointestinal tract damage (nausea, vomiting, abdominal pain) are noted, but bloody diarrhea is not characteristic.

Symptoms of thrombotic thrombocytopenic purpura

The majority of patients with thrombotic thrombocytopenic purpura develop a single acute episode of the disease that does not recur after successful therapy, but more recently chronic chronic recurrent forms of thrombotic thrombocytopenic purpura have been observed. An acute episode is preceded by a prodrome, usually occurring in the form of an influenza-like syndrome; In rare cases, diarrhea is noted. Thrombotic thrombocytopenic purpura is characterized by high fever, dermal purpura (practically in all patients), which can be combined with nasal, uterine, gastrointestinal hemorrhages.

CNS lesion is the dominant symptom of thrombotic thrombocytopenic purpura, noted in 90% of patients. In the debut of the disease, the most frequent are intense headaches, drowsiness, inhibition, there may be impairment of consciousness, which is combined with focal neurological symptoms. In 10% of patients develops a coma. In most cases, these symptoms are unstable and disappear within 48 hours.

The kidney damage is manifested by moderate urinary syndrome (proteinuria not more than 1 g / day, microhematuria) with impaired renal function. Oliguric acute renal failure is rare, but this or that degree of renal insufficiency is present in 40-80% of patients. In addition to affecting the central nervous system and kidneys, heart, lungs, pancreas, and adrenal glands can develop.