Laboratory Studies. Vaquez’ disease (erythremia)

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Laboratory Studies

· Rapidly progressive severe hyperbilirubinemia or prolonged hyperbilirubinemia

· Positive maternal antenatal antibody findings and/or diagnosis of anemia or fetal hydrops

· Positive neonatal direct Coombs test (direct antiglobulin test)

· Hemolysis on blood film findings

The severity of hematologic abnormalities is directly proportional to the severity of hemolysis and the extent of hematopoiesis. The following abnormalities are observed on CBC count findings:

· Anemia:

· Increased nucleated RBCs, reticulocytosis, polychromasia, anisocytosis, spherocytes, and cell fragmentation

o The reticulocyte count can be high 40%

o The nucleated RBC count is elevated and falsely elevates the leukocyte count, reflecting a state of erythropoiesis.

o Spherocytes (< 40%) are more commonly observed in cases of ABO incompatibility.

o In severe hemolytic disease, schistocytes may be observed, reflecting ongoing disseminated intravascular coagulation.

o Abnormally elevated mean cell hemoglobin concentration (MCHC)

Erythremia (true polycythemia, vera) – is a chronic leukemia with impairment at the level of a stem cell or a precursor-cell of myelopoiesis. It is characterized by total hyperplasia of medullary cells with abnormal tumor proliferation of erythroid, myeloid and megakaryocyte germs (panmyelosis), mainly of an erythroid series (primary absolute erythrocytosis) – the erythrocyte count irreversibly increases, while the level of erythropoietine in the blood and urine is low.

The disease is considered to have two clones of erythroid precursors: that is dependent on erythropoietine and an independent one, erythroid colonies of which grow without erythropoietine. However the characters of differentiation in erythroid cells are preserved.

The onset of the disease is gradual, the skin and mucous membranes get a cherry-red color with a cyanotic shade, it is marked by dizziness, headaches, noise in the ears, injection of vessels of the retina and conjunctiva («rabbits’ eyes»), BP sharply elevates.

The symptoms are explained by increasing of the circulating blood mass, increased number of blood cells (polycytemic hypervolemia), that causes blood viscosity, slowing down of the blood flow, impairment of hemodynamics. There develops hypoxia, hepatomegaly and splenomegaly.

The hemogram reveals erythrocytosis (up to 6.0 – 12.0. 1012/l), hypochromia (high velocity of iron consumption, decreasing of its reserves), reticulocytosis, the hematocrit index is 60 – 80%; neutrophile leukocytosis with a shift of the leukocyte formula to the left, thrombocytosis with giant shapes. The content of hemoglobin increases up to 180 – 200 g/l; there is noted polychromasia, anizocytosis, basophile granularity of erythrocytes, normoblastosis, toxic granularity of neutrophiles. ESR is reduced.

There occur transformation of the yellow bone marrow into the red one; the myelogram shows increasing of the elements number of an erythrocyte, granulocyte and megakaryocyte series.

 Basic complications: a thrombohemorrhagic syndrome, coronary and cerebral ischemia, strokes, central paralyses, blindness (thrombosis of retinal veins). Thromboses of abdominal veins give symptoms of «acute stomach». In the terminal stage erythremia usually transforms into myelofibrosis and pancytopenia develops.

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