Hemophagocytic syndrome? This starts with the platelet review that day

Recently, a patient with suspected hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, was found through reexamination of platelets. Through communication with the clinic, we can improve our understanding of the disease. This is a good case, which is reported as follows. < / P > < p > if we only observe the platelet data, it is really confusing: the difference between the results of laser and that of impedance method is nearly 4 times, and the instrument automatically selects the detection result of laser method, 111 × 109 / L. I carefully checked the histogram and scatter diagram, and found that the platelet histogram was obviously abnormal, and the tail was obviously upwarped, indicating the existence of high-end interference, and the scatter diagram of plt-o channel was obviously abnormal. There was an obvious abnormal area at the intersection of the scatter diagram of platelet and Y axis, which was rarely seen in normal times. Therefore, it is suggested that colleagues make blood smears, and then determine the true value of platelets after microscopic staining. < p > < p > thinking about this case, I went to work early in the afternoon to find the specimen in the morning. After standing for more than two hours, the plasma appearance was cloudy, as shown in Figure 3 below. < / P > < p > find the blood smear picture made by colleagues in the morning, read the film on di60, and see the red blood cells under the oil microscope field as shown in Figure 4: the average number of platelets in each oil microscope field is 2-3, and the platelet count is roughly estimated to be about 25-38 × 109 / L, which is basically consistent with the colleague’s report of 35 × 109 / L. < / P > < p > so far, the platelet reexamination is over, and it has been completed perfectly. But the histogram, scatter diagram and occupational sensitivity of abnormal platelets suggest that there must be some clue behind the specimen. Then log in to LIS workstation and check the basic information and other test reports of the patient in detail, as shown in Figure 5. < p > < p > in the biochemical results, blood lipid was significantly increased, triglyceride TG: 6.89mmol/l, LDH: 507iu / L, also moderately increased, the chylous state of the specimen was clearly marked in the remarks. Fibrinogen FB: 0 g / L on July 27. < / P > < p > decreased blood cells, thrombocytopenia, hyperlipidemia, low fibrin? Is the patient hemophagocytic syndrome? Serum ferritin SF was added to the patient, and the result was: 1359ng / ml, which further confirmed my judgment: This is probably a patient with hemophagocytic syndrome. As a result, I quickly contacted the clinician and learned that the patient was a 2-year-old child with acute myeloid leukemia. After 3 months of stem cell transplantation, he was hospitalized due to fever and was initially diagnosed as pulmonary infection. At present, the patient is still having fever, and the treatment effect is not good. Due to respiratory failure, he is admitted to the intensive care unit and wears a ventilator. After understanding, clinicians have been in a state of significant increase in blood lipids of children in recent years, and feel very confused. < / P > < p > after understanding these basic conditions, I combined with the 2004 edition of the diagnostic criteria for hemophagocytic syndrome [1], when meeting the following or 5 of the following 8 indicators, any one can be diagnosed. < p > < p > 4 √ hypertriglyceridemia and low fibrinogen: TG & gt; 3 mmol / L or higher than 3 standard deviations of the same age, FB & lt; 1.5g/l or 3 standard deviations lower than the same age; < / P > < p > discuss with the doctor. In view of the fact that the fever is more than 7 days, the number of peripheral blood line 2 blood cells decreases, triglycerides increase and low fibrinogen, serum ferritin: 1359ng / ml, it is recommended that clinicians consider that children can have secondary hemophagocytic syndrome, and ask them to improve bone marrow puncture and NK cell activity test The bed doctor agreed with my suggestion and improved the relevant examination. < p > < p > three days later, the results of bone marrow report came out, and no abnormality was found. So far, the child has met the 4 diagnostic criteria for hemophagocytic syndrome, but there is no description of hemophagocytic cells found in the expected bone marrow report, which is undoubtedly a heavy blow to my diagnosis direction. Is my diagnosis wrong? Or are there no hemophagocytes in the bone marrow? Or are hemophagocytes not found? This kind of doubt confused me, but the blood lipid of the child was significantly increased, the serum ferritin was significantly increased, the peripheral blood two lines were significantly reduced, the fibrinogen result was once 0, intuitively, these evidences all point to hemophagocytic syndrome. As a result, I found the bone marrow smear, and I searched it carefully under the microscope. My efforts paid off. I found two suspicious cells in the bone marrow smear, but they were not. Identification points: when the proliferation of bone marrow cells is active / slightly reduced, it is necessary to see typical phagocytes to confirm. In the figure below, there is no nucleus of phagocyte itself in the first picture, and there are no two points in the second picture; when myelodysplasia is extremely reduced, a large number of hemopoietic cells may have been swallowed by phagocytes, then a large number of activated histiocytes and hemopoietic cells can be seen in bone marrow The number of cells is extremely reduced or even invisible, which is not the case in this patient. < / P > < p > there are generally 5-7 bone marrow slices sent for examination after bone marrow puncture. Can typical cells be found in other bone marrow slices? Only one bone marrow slice was stained immediately, and two suspected hemophagocytes were found under the microscope within two minutes. After comparison of the above identification points, these two cells can only be identified as tissue cells, not haemophiles. The former belongs to normal cell metabolism, and we occasionally see them in non malignant diseases with active bone marrow proliferation in our daily work. At this point, we have communicated with the clinicians all the examination conditions of the patients, hoping to further exclude HLH. The clinicians are also confused about why a patient with bone marrow transplantation has the phenomenon of suspected HLH. < / P > < p > in order to further clarify the diagnosis of patients, the next step of treatment plan should be made by clinical consultation. Relevant literature has been consulted since 2012, 2] Since then, more and more literatures have reported that dyslipidemia after allogeneic hematopoietic stem cell transplantation and autologous bone marrow cell transplantation is a common but easily overlooked event. More and more evidence shows that early intervention is needed to prevent the occurrence of cardiovascular disease in the future and improve the long-term survival of patients. What’s more, it is worthy of attention that these patients should pay attention to The use of drugs may play a role in the regulation of graft-versus-host disease, so clinical attention should be paid. < / P > < p > can we still diagnose HLH in this special patient with dyslipidemia? In addition, the patient’s ferritin increased, and what is the reason? According to the literature [3,4], 42.2% of children with acute leukemia developed ferritin & gt; 350ng / ml after transplantation, One of the influencing factors is that the primary disease is acute myeloid leukemia, and the other is GVHD. The patient was admitted to the hospital because of pneumonia and respiratory failure. Whether it is caused by GVHD needs further examination and confirmation. However, there is evidence that has fully explained all the detection indicators of suspected HLH. After we contacted the clinic once again, we confirmed the next treatment plan. We would like to express our thanks for this and hope that we can help to further observe whether some indicators have the same or different performance after umbilical cord blood transplantation. < / P > < p > the Enlightenment of this case is: the application of diagnostic criteria is flexible and changeable, and sometimes the diagnostic criteria are no longer the standards according to the different conditions of patients. Dare to question, carefully verify. Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, is an excessive inflammatory response syndrome caused by primary or secondary immune abnormalities. The abnormal immunoregulation is mainly caused by the abnormal activation and proliferation of lymphocytes, monocytes and macrophages, and the secretion of a large number of inflammatory cytokines. The main clinical features were persistent fever, hepatosplenomegaly, pancytopenia, high triglyceride, low fibrinogen, high serum ferritin, and hemophagocytosis in bone marrow, liver, spleen and lymph nodes. Due to different trigger factors, HPS can be divided into two categories: primary and secondary. The former mainly occurs in children, most of them have family history, and are accompanied by genetic abnormalities; the latter can occur at all ages, mostly caused by infection, tumor and autoimmune diseases. The most common laboratory characteristics of HPS are: peripheral blood abnormalities are hemocytopenia, often two-line or whole blood cytopenia. The abnormal liver function was mainly manifested by the increase of serum transaminase, bilirubin, lactate dehydrogenase, and the decrease of albumin level with the increase of serum triglyceride level. The coagulation function was abnormal. The coagulation time was prolonged and the fibrinogen content was significantly decreased. Serum ferritin increased significantly, which can be used as a marker of disease activity. < / P > < p > first of all, we should strictly implement the re inspection rules. In daily work, if abnormal blood routine results are found, don’t be afraid of trouble. Smear and microscopic examination should be carried out in time instead of relying on machine results only. In the process of routine blood test, the histogram and scatter diagram of platelets were found to be abnormal. The results of impedance method and flow laser staining method were nearly 4 times different. After reexamination under the microscope of platelets, it was confirmed that the results of laser method were wrong, and the results of impedance method were correct. This overturned the recognition of the high reliability of laser counting results. In addition, it is necessary to be highly sensitive to common diseases, integrate biochemical, immune and clinical cases, and communicate with doctors in a timely manner. In this case, through the surface phenomenon of decreased platelet count and abnormal scatter plot and histogram in blood routine examination, the biochemical results and electronic medical records were actively reviewed. The laboratory characteristics of significant increase in blood lipid and significant decrease in fibrinogen were found. Hemophagocytic syndrome was highly suspected. Serum ferritin test was actively performed to confirm the inference, and actively contacted with clinical practice. Finally, he actively participated in clinical diagnosis and treatment, boldly gave suggestions for the next clinical step, and gave appropriate hierarchical diagnosis report, which reflected a high professional value and responsible attitude of a laboratory physician. According to the abnormal platelet count in this case, a layered report was given. A possible diagnosis direction was suggested in Level 2 [2], and appropriate suggestions for further improving bone marrow puncture and NK cell activity detection were given in Level 3. In the actual blood cell analysis process, fully implemented the re examination rules, and further deepened the application in the actual work, which was fully affirmed by clinicians. < / P > < p > therefore, in the process of communication with the clinic, we should actively seek truth and boldly infer. We should actively communicate with the clinic and escort the patient’s health with professional knowledge. The value of the laboratory is in our own hands. [2] hematology and humoral science group, society of laboratory medicine, Chinese Medical Association. Guidelines for standardization of blood cell analysis reports [J]. Chinese Journal of laboratory medicine 114452 20200331 00343 < A= https://luanban.com/small-habits-of-pregnancy-not-only-affect-the-health-of-pregnant-mother-but-also-delay-the-development-of-the-fetus-dont-ignore/ target=_ blank>Small habits of pregnancy not only affect the health of pregnant mother, but also delay the development of the fetus, don’t ignore