Paroxysmal cold hemoglobinuria

Introduction to paroxysmal cold hemoglobinuria Paroxysmalcoldhemoglobinuria (PCH) is a rare disease characterized by hemoglobinuria that occurs suddenly after systemic or local cold. The factors that induce hemolysis are mostly acute after cold, but not obvious. . After a few minutes or hours, a brief chill, fever (up to 40 ° C), general weakness, abdominal discomfort, back and lower extremity pain, nausea, vomiting. The first urine was followed by hemoglobinuria, which was dark red or black. basic knowledge The proportion of illness: the incidence rate is about 0.002%-0.003% Susceptible people: no specific population Mode of infection: non-infectious Complications: thrombosis

Paroxysmal cold hemoglobinuria

(1) Causes of the disease

Virus, mycoplasma, spirochete infection.

(two) pathogenesis

It has been determined that the hemolysis of this disease is caused by a 7S IgG cold antibody (Donath-Landsteiner antibody, called DL antibody) in the blood. When the temperature drops below 20 °C, the cold antibody binds to the red blood cells and activates complement. When the temperature is raised to 37 ° C, the antibody falls off, but the order of complement activation is completed, that is, hemolysis occurs, and the DL antibody is a hemolysin, but there is also some agglutination.

Paroxysmal cold hemoglobinuria prevention

Paroxysmal cold hemoglobinuria complications Complications thrombosis

1. Infection: Patients with paroxysmal cold hemoglobinuria are susceptible to various infections, especially respiratory and urinary tract infections, which can induce hemoglobinuria. In China, severe infections are often the leading cause of death in patients with paroxysmal cold hemoglobinuria.

2. Thrombosis: Thrombosis in different parts accounts for 23% to 50% of paroxysmal cold hemoglobinuria in Europe and America, and is the leading cause of death in patients with paroxysmal cold hemoglobinuria in these areas.

Paroxysmal cold hemoglobinuria symptoms common symptoms proteinuria hemoglobinuria abdominal discomfort nausea weakness cold war low back pain low molecular proteinuria

Most of the factors that induce hemolysis are acute onset after cold, but there are also those who are not obvious, appear in a few minutes or hours, short chills, fever (up to 40 °C), general weakness, abdominal discomfort, pain in the lower back and lower limbs, nausea , vomiting, and then the first urine is hemoglobinuria, dark red or black, from symptoms to hemoglobinuria for a few minutes to 8h, acute systemic reactions and hemoglobinuria can disappear within a few hours, it can last for several days, The patient has splenomegaly and hyperbilirubinemia. He may have hemosiderin after repeated attacks. The systemic symptoms are more significant than CAS. The PCH patients caused by syphilis may have Raynaud syndrome.

Paroxysmal cold hemoglobinuria examination

1. Peripheral blood is severe when the anemia is severe and progresses rapidly. The reticulocytes in the acute phase are usually reduced, but eventually there will be an increase in reticulocytes. The surrounding blood may have different red blood cells and deformities, and there are spherical red blood cells, red blood cell fragments, and alkalophils. Dotted and multi-stained red blood cells, even young red blood cells can appear, with a brief leukopenia at the beginning, followed by leukocytosis.

2. The hemosiderin test is positive.

3. The hot and cold hemolysis test (DL test) is positive.

4. Coombs test positive C3 type.

Diagnosis and diagnosis of paroxysmal cold hemoglobinuria

Diagnostic criteria

In addition to typical clinical manifestations, positive cold and cold hemolysis test is the basis for the diagnosis of this disease.

1. Diagnostic criteria and basis

(1) Acute onset after cold, severe anemia, hemoglobinuria.

(2) Positive heat and cold hemolysis test.

(3) Coombs test positive C3 type.

2. Diagnostic evaluation

(1) PCH is a rare disease with a clear cause: it is more common in children, so patients with hemoglobinuria should first exclude other diseases, and then consider PCH.

(2) Direct Coombs test: It can be positive in the onset of hemoglobinuria, and the interval is very negative after the onset. The time of specimen delivery should be noted. The positive rate of antiserum test in the Coombs test should be improved.

(3) Paroxysmal cold hemoglobinuria has primary and secondary points: it was thought to occur only in syphilis in secondary diseases, and in recent years it is thought to be related to viral infectious diseases, such as infectious mononuclear cells. Indications, mycoplasmal pneumonia, measles, mumps, viral hepatitis, etc., should be given adequate attention.

Differential diagnosis

1. Paroxysmal nocturnal hemoglobinuria is mostly a chronic course, recurrent hemoglobinuria, laboratory test Ham test (+), specific antibody CD55, CD59 negative cells increased.

2. Warm antibody type autoimmune hemolytic anemia may have similar clinical manifestations and transient hemoglobinuria in the acute onset, but the incentives are different, the cold and hot hemolysis test is negative.

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