What is pancytopenia

Some diseases are very unfamiliar to people. Few people probably know what pancytopenia is. This is a blood disease. From the name, it means that the blood cells in the whole body are constantly decreasing. It seems that this disease is actually very serious and will definitely have a great impact on the body. However, no matter what disease it is, you must actively cooperate with the treatment. Only in this way can you get better faster.

Pancytopenia, also known as aplastic anemia (AA), is a pancytopenia syndrome caused by bone marrow hematopoietic failure. It is divided into two major categories: congenital and acquired, with acquired types accounting for the vast majority. Congenital aplastic anemia is very rare, and its main type is Fanconi anemia. Acquired aplastic anemia can be divided into primary and secondary types. The former is of unknown cause, accounting for about 50% of acquired aplastic anemia; it can also be divided into acute and chronic types according to different comprehensive classifications of clinical manifestations, blood pictures and bone marrow pictures. Foreign countries divide severe aplastic anemia according to the severity. The latter classification standard requires that the blood picture has two of the following three items: ① The absolute value of neutrophils and the degree of bone marrow cell proliferation are lower than 25% of the normal level. If the clinical manifestations of aplastic anemia are mainly anemia, bleeding, and infection. The severity of clinical manifestations depends on the degree of hemoglobin, leukocytes, and thrombocytopenia, and is also related to the clinical type.

1. Acute aplastic anemia

The characteristics of acute aplastic anemia are rapid onset, rapid progression, and short course. Anemia is often not obvious in the early stage of the disease, but as the disease progresses, anemia progressively worsens, and there are often obvious symptoms such as fatigue, dizziness, and palpitations. Even with massive blood transfusions, anemia is difficult to improve. Bleeding and infection are often the main symptoms at the onset of the disease. Bleeding occurs in almost every case, and the bleeding sites are widespread. In addition to surface bleeding such as the skin, mucous membranes (oral cavity, nasal cavity, gums, and conjunctiva), there is often bleeding in deep organs, such as blood in the stool, blood in the urine, vaginal bleeding, fundus bleeding, and intracranial hemorrhage, the latter of which often endangers the patient's life. More than half of the cases are infected at the onset, with oropharyngeal infection, pneumonia, skin furuncle, intestinal infection, and urinary tract infection being the most common. In severe cases, sepsis may occur. The most common pathogenic bacteria are Escherichia coli, Pseudomonas aeruginosa and Staphylococcus aureus. Infection often worsens bleeding and often leads to death.

2. Chronic aplastic anemia

The characteristics of chronic aplastic anemia are slow onset, slow progression and long course. Anemia is the first and main manifestation. Blood transfusion can improve anemia symptoms such as fatigue, dizziness, and palpitations. Bleeding is generally mild, mostly superficial bleeding such as skin and mucous membranes, and deep bleeding is very rare. During the course of the disease, there may be mild infections and fever, most of which are respiratory infections and are easier to control. If the infection is severe and the high fever persists, it often leads to worsening bone marrow failure and turns into severe aplastic anemia.

3. Aplastic anemia-related diseases

(1) Paroxysmal nocturnal hemoglobinuria (PNH)/aplastic anemia: PNH and aplastic anemia are strongly associated with each other. The two diseases often occur simultaneously or successively in the same patient. They have similar clinical features, including pancytopenia and hypoplastic bone marrow. The disease has a regional tendency, with a high incidence in Asia. PNH is responsive to immunosuppressant treatment. PNH is characterized by intravascular hemolysis, venous thrombosis, and bone marrow hematopoietic failure, and many patients die from thrombosis rather than bleeding complications.

(2) Hepatitis/aplastic anemia syndrome: Aplastic anemia after acute viral hepatitis is not uncommon, and hundreds of cases have been reported to date. Among patients with aplastic anemia reported in the West, 2% to 9% had a history of hepatitis, and the proportion may be higher in Asia. Although viral hepatitis may sometimes be associated with mild cytopenias, severe pancytopenia and hypoplastic bone marrow are uncommon and are estimated to account for 2% of hepatitis B in children. Among patients with fulminant seronegative hepatitis leading to liver failure, one-third will eventually develop aplastic anemia. Post-hepatitis aplastic anemia has the following characteristics:

① It often occurs within 1 to 2 months after viral hepatitis. Severe pancytopenia occurs during the recovery period of inflammation. Mild blood cell reduction may occur during the inflammatory period of viral hepatitis, such as granulocyte and thrombocytopenia, macrocytosis, atypical lymphocytosis, etc., which are similar to the manifestations of mild aplastic anemia. Its prognosis is extremely poor, with a mortality rate of up to 90% within 1 year;

② The virus that causes post-hepatitis aplastic anemia is still not very clear, and almost all studies have shown that the virus is non-A, non-B, non-C, and non-G hepatitis virus. It is common for patients with aplastic anemia to have concurrent hepatitis C and hepatitis G viral hepatitis, which is often believed to be caused by repeated blood transfusions rather than the cause of aplastic anemia. ③ Seronegative acute viral hepatitis is clinically significantly different from hepatitis C, that is, parental exposure is not a risk factor, the patient's acute phase liver function abnormalities are very severe, and late complications are common. For hepatitis/aplastic anemia syndrome, allogeneic bone marrow transplantation should be the first choice. For patients with obvious markers of immune activation, intensive immunosuppressive therapy is often effective.