Hemoglobinemia

Many people in their daily lives know that once hemoglobinemia occurs, the harm will be quite great, and there is no clear goal related to this in normal times. This is a myeloproliferative disease and there are no clear preventive measures, but it also requires corresponding diagnosis. Only a reasonable diagnosis can prevent the disease from worsening. I want to understand the relevant care plans and risk factors so that I can know what the concept is.

Pathological etiology The cause of this disease is still unclear. In most patients, the EPO levels in plasma and urine not only do not increase, but decrease significantly. Cell culture showed that the number, affinity and expression of EPO receptors in erythroid progenitor cells of PV patients were no different from those of normal people, and sequence analysis of the EPO gene encoding it also found no abnormalities. The above results show that the EPO receptor is not involved in the pathogenesis of the disease. Modern studies have shown that PV is not an excessive proliferation of normal stem cells, but is caused by abnormal clonal proliferation of a single cell origin.

Disease Diagnosis 1. Secondary and relative erythrocytosis Secondary erythrocytosis is common in the following two situations: First, tissue hypoxia or renal ischemia-hypoxia caused by increased EPO secretion, leading to compensatory increase in red blood cells, which can be seen in mountain sickness, congenital heart disease with right-to-left shunt, chronic lung disease, methemoglobinemia, carboxyhemoglobinemia caused by smoking, etc. The patients' blood oxygen saturation is mostly reduced. The other is polycythemia caused by the autonomous secretion of erythropoietin or erythropoietin-like substances by renal tumors and other endocrine tumors, which is seen in Wilms tumor, liver cancer, cerebellar tumor, diencephalic tumor, kidney cancer, uterine tumor, etc. Relative polycythemia is caused by a relative increase in red blood cell volume due to a decrease in plasma volume. The peripheral red blood cells, hemoglobin and hematocrit increase, but the systemic blood cell volume is normal. It is common in temporary fluid loss such as dehydration and burns, and chronic relative erythrocytosis (Gaisbock syndrome) caused by smoking, drinking, anxiety and hypertension. See Table 1 for specific identification.

2. Chronic myeloid leukemia (CML) PV patients often have splenomegaly and granulocytosis, and in the late stage, the number of immature granulocytes in peripheral blood may increase, so it needs to be differentiated from CML. The neutrophil alkaline phosphatase score of PV patients is elevated, the Ph1 chromosome and bcr/abl mRNA are negative, while the opposite is true for CML patients. Recent studies have found that CFU-E can also be formed spontaneously in patients with CML, so endogenous CFU-E cannot be used to differentiate between PV and CML.

3. Myelofibrosis The clinical manifestations of PV have many similarities with myelofibrosis. Myelofibrosis may also occur in the late stage of PV. The two are mainly differentiated by medical history and bone marrow biopsy. Myelofibrosis bone marrow pathology shows a significant increase in fibrous tissue, while PV mainly manifests as extramedullary hematopoiesis. Myelofibrosis is only combined with late stage, and the lesion range is small and the degree is relatively mild.