Introduction
This is a case where a disease is hidden in one case and is very easy to miss diagnosis. The patient is very young and has pulmonary embolism without obvious causes. All seemingly reasonable logical inferences were found through layers of careful investigation. After several hospitalizations, it is hard to predict that after being discharged from the hospital, it was found that the disease was hidden in the disease. This extremely hidden rare disease is a chronic progressive inflammatory disease with unknown cause and mainly damage to lymph node , soft tissue and salivary gland . It is specialized in East Asians, middle-aged and young men. Looking back at the two diseases that patients have suffered, there is always an important clue that is the "initiator" of the disease. Is it really the "fuse"? What prompts should its appearance bring to our diagnosis?
9-year-old boy developed from pleural effusion to severe pulmonary embolism and was admitted to intensive care unit for only more than a week. The onset was urgent and the progress was fast. What is the reason why the pleural effusion was not absorbed after discharge?
patient was born in Changde City, Hunan Province. He studied at a university when he was on the disease. He was a 19-year-old boy. The main complaint found that "pleural effusion for more than 1 month" was admitted to our hospital on October 24, 2018. He claimed that he had paroxysmal cough and sputum without obvious causes. The local hospital considered "chronic pharyngitis" and gave amoxicillin to fight infection. The symptoms improved compared with the previous period after treatment.
On September 6, 2018, the patient developed left chest pain and paroxysmal colic, mainly precordial area and shortness of breath. The local hospital's chest radiograph examination was considered "bronchiolitis?", and then visited the emergency department of Xiangya Third Affiliated Hospital. The next day, the patient developed fever, with a maximum body temperature of 39.5℃. Both lung CT showed: double pneumonia, and a small amount of pleural effusion on the left. Levofloxacin injection was given; the routine blood results on September 8 showed: WBC16.76*10^9/L, platelets 31*10^9/L; on September 10, swelling below the knee joint of the right lower limb, no obvious skin cyanosis, and no pain; on September 13, swelling below the knee joint of the right lower limb was found with discomfort below the knee joint of the right lower limb, accompanied by chest pain and worsening of . The CTA of the pulmonary artery showed: bilateral pulmonary artery embolism, color ultrasound of the right lower limb showed: embolism of the right superficial femoral, deep femoral, posterior tibial and popliteal vein, and the right large saphenous vein was insufficiency. He was immediately admitted to the intensive care unit of Xiangya Affiliated Hospital for hospitalization.
From the discovery of pleural effusion, it was considered chronic pharyngitis, to chest pain, shortness of breath, bronchitis, to fever, pneumonia, and then to lower limb swelling, chest pain worsening, pulmonary artery embolism, and entering the intensive care unit. The whole process of was only more than a week, and the disease occurred rapidly and progressed quickly, but we need to pay attention to one point, why does such a young man have such a serious pulmonary embolism? In terms of treatment, during the hospitalization of the intensive care unit of Xiangya Affiliated Hospital, the patient was diagnosed with IgE 1730IU/ml, anti-cardiolipin IgA (+), anti-cardiolipin IgG (+), protein C activity 88%, protein S activity 0.2%↓, and was diagnosed with: 1. Acute pulmonary embolism, type I breathing failure, pleural effusion; 2. Community-acquired pneumonia; 3. Right lower limb venous thrombosis ; 4. Thrombocytopenia (the cause is to be investigated), and the patient occasionally had hemoptysis, and was given " meropenem combined with teicolanin (9.14-9.23)", "levofloxacin" anti-infection, "rivaroxaban" anticoagulation, and combined with pleural hydration drainage. Pleural fluid routine: red color, turbid transparency, total number of cells 110,200, nucleated cells 2990, mononuclear 52.7%, multinuclear 45%; pleural fluid biochemistry: total protein 44.4, albumin 26.7, chlorine 109.7, glucose 6.44, ADA 0, LDH 484; pleural fluid CEA 0.591, no bacteria were found in smear + G staining, acid-resistant staining was negative, a large number of shedded and degenerative mesothelial cells and neutrophils were seen in the pleural fluid picture, and no tumor cells were found.
Starting from September 16, the patient had no fever or chest pain, and the symptoms of shortness of breath also improved significantly, and the cough and sputum were relieved; on September 27, three-dimensional imaging of the lungs: a large amount of pleural effusion in the middle of the right side, part of the right lung swelled incompletely, left pneumonia was absorbed more than anteriorly, and left pleural effusion decreased compared with anteriorly. Because the patient's symptoms improved significantly compared with the previous one, he was discharged from the hospital on October 6. During the period, the color ultrasound of the pleural effusion was checked regularly, but the effusion was not significantly absorbed (Table 1).
Table 1. The amount of pleural effusion shown in color ultrasound of the pleural effusion
was admitted to the hospital again for diagnosis and rebirth of sinus. What clues were found after further examination? Is the unabsorbed pleural effusion related to eosinophil eosinophil euphratica?
In order to seek further treatment, on October 23, 2018, the patient came to our hospital for treatment. The blood test routine was: the percentage of eosinophils was 9.5%, and the absolute value of eosinophils was 0.91*10^9/L. The liver and kidney function is normal. The outpatient clinic is included in our department with "pleural effusion". At this time, the patient had no cough, sputum, fever, chest pain and shortness of breath, and his diet and sleep were normal, and his stools were normal.
Tracking past history learned that the patient had soreness and discomfort on the medial side of the right ankle after playing basketball around August 21, and the discomfort extended to the top of the heel. On September 1, after applying (not rubbing and pressing) external medicine for promoting blood circulation and removing blood stasis, a 3x5cm-sized ecchymia appeared on the inner side of the right ankle joint with swelling, but after ice and applied external medicine, the ecchymia and swelling significantly subsided. In the past two years, the patient's allergic dermatitis has repeatedly occurred, but no allergic was found. The patient has a history of allergic to cefomexime and a history of allergic to roxithromycin , but there is no special one. The patient is an only child, unmarried, and denies his history of smoking and drinking. The parents are alive and deny their familial genetic history.
After being admitted to our hospital, the first physical examination was performed:
Body temperature was 36.7℃; pulse 125 times/min; breathing 20 times/min; blood pressure 135/87mmHg. The patient's chest was not deformed, the right lower lung tremor was weakened, the right lower lung percussion sound was sounded, the right lower lung breathing sound disappeared, and no dry and wet rales and pleural friction sounds were heard. There was no bulge in the precordial area, the apical pulsation was 0.5cm in the midline of the left clavicle of the fifth intercostal region, and did not touch tremor , the heart boundary did not expand, the heart rate was 125 beats/min, the rhythm was smooth, and there was no significant enhancement or weakening of the heart sounds of . There was no pathological noise in each valve auscultation area, and there was no special one. The auxiliary examination of
is:
On September 13, 2018, color ultrasound of the deep vein of both lower limbs showed that the right superficial femoral, deep femoral, posterior tibial and popliteal vein emboli were formed; the right large saphenous vein valve was insufficiency.
September 13, 2018 pulmonary artery CTA showed: bilateral pulmonary artery embolism.
Bone marrow aspiration on September 27, 2018 showed that myeloproliferative activity, the proportion of bone marrow and peripheral eosinophils is relatively high, the distribution of megacculus accounts for 4/18 of the plate production, and platelet distribution is rare.
team analyzed the patient's condition, summarized the case characteristics: 1. Young male, course of the disease 2 months; 2. Onset with cough, shortness of breath, and fever; 3. Past: history of rash; 4. Auxiliary examination: elevated eosinophils; pulmonary artery CTA indicates bilateral pulmonary artery embolism, bipneumonia, bilateral pleural effusion, more on the right side, and pleural effusion indicates exudate; color ultrasound indicates deep venous thrombosis in the right lower limb.
Thinking questions: So far, what are the diagnostic and treatment ideas for this patient? For example, it is necessary to clarify whether the nature of pleural effusion is exudate or leaky fluid? For example, why does the patient's pleural fluid still not absorb after inflammation is controlled? What possibilities need to be considered? What kind of treatment plan should be chosen while clarifying the diagnosis and treatment ideas? What further inspection items should be carried out?
According to the performance of tuberculous pleural effusion , considering that the possibility of pleural effusion caused by infection is high, and the pleural effusion is still not absorbed after inflammation control. Considering that pleural effusion may be caused by pulmonary embolism
The following are diagnostic and treatment ideas (I):
, diagnosis and treatment ideas. patients are young men. First of all, the diagnosis of bilateral pleural effusion is clear based on lung CT and color ultrasound. Bilateral pleural effusion is more common in leaky fluid. Depending on the cause, exudate can also manifest as bilateral.
The second step is to clarify the nature of pleural effusion, whether it is exudate or leaky fluid? determines whether pleural effusion is exudate. The most commonly used standard in clinical practice is the Light standard: 1. The ratio of pleural effusion protein to serum total protein is >0.5; 2. The ratio of pleural effusion LDH to serum LDH is >0.6; 3. The upper limit of the laboratory normal value of pleural effusion LDH >2/3 serum LDH. If one or more of the above one is the exudate, the accuracy can reach 93%-96%. However, it should be noted that pleural effusion concentration in patients with congestive heart failure after using diuretics will lead to an increase in total protein, LDH and fat content. The Light standard may classify this part of the leaking fluid as exudate fluid. This patient had a pleural effusion examination in an external hospital and was judged as exudate according to the Light standard.
Step 3, find out the cause of pleural effusion. From the perspective of common diseases and frequent occurrence, the most common diseases of exudative pleural effusion are malignant tumors, paraplegia effusion, and infection. The less common causes include pulmonary embolism, rheumatoid arthritis and other autoimmune pleurisy, benign asbestos pleural effusion, pancreatitis , after myocardial infarction, after coronary artery bypass transplantation, rare causes include chlora syndrome and other lymphatic abnormalities. For example: lymphangioleiomyomatosis, drugs (such as methotrexate , amiodarone , phenytoin , etc.), fungal infections. Based on the patient's situation, no placeholder lesions were found, and the pleural effusion CEA was not high, so malignant tumors could be basically ruled out; the patient's pleural effusion accounted for 45%, ADA was not high, accompanied by high fever and inflammation indicators, which did not meet the characteristics of tuberculous pleural effusion, and was considered to be highly likely to cause pleural effusion due to infection. However, the patient also has pulmonary embolism. If the pleural effusion is not absorbed after inflammation is controlled, the possibility of pleural effusion caused by pulmonary embolism should be considered.
. Ideas for diagnosis and treatment of pulmonary embolism. patients had pulmonary embolism symptoms such as cough, shortness of breath, chest pain, etc. The pulmonary artery CTA was used to clarify the double pulmonary artery embolism. The pulmonary artery CTA was a confirmed examination of acute pulmonary embolism, and there was also deep venous thrombosis in the right lower limb, which could be diagnosed as venous thromboembolism (VTE).
For patients with VTE, a cause-required examination is also required. Any factors that can cause venous blood stasis, vascular endothelial injury and blood hypercoagulation status (Virchow three elements) are risk factors for VTE, including hereditary and acquired categories.
Heraldical factors: caused by genetic variation, and are often caused by repeated arteriovenous thrombosis as the main clinical manifestations. If patients aged 50 years old have recurring VTE or tend to have familial morbidity without obvious causes, they should be alert to the existence of thromboproliferative disease.
Acquisitive factors: Acquisitive risk factors refer to various pathophysiological abnormalities that are prone to VTE acquired, mostly temporary or reversible. For example: surgery, trauma, acute internal medicine diseases (such as heart failure, respiratory failure , infection, etc.); certain chronic diseases (such as antiphospholipid syndrome, nephrotic syndrome , inflammatory bowel disease, myeloproliferative disease , etc.).
Malignant tumors are an important risk factor for VTE, but the VTE risk of different types of tumors is different. malignant tumors in pancreatic , craniocerebral, lung, ovary and hematologic system are considered to have the highest VTE risk, and the risk of VTE during the active period of malignant tumors is increased. Some VTE patients cannot identify the risk factors after relatively complete testing methods, which are called idiopathic VTE. For this patient, young people, there is no obvious cause of pulmonary embolism, and the possibility of thrombosis should be considered. Anticoagulant proteins such as antithrombin , protein C, protein S, etc. should be checked for antiphospholipid syndrome related tests. If necessary, improve gene detection related to thrombosis .
For patients with confirmed acute pulmonary embolism, risk stratification is required. First, the degree of risk is distinguished based on hemodynamic . Hemodynamic unstable people are defined as high-risk, and hemodynamic stable people are defined as non-high-risk. This patient was in a non-high risk group. Hemodynamic stable patients distinguish them into medium-hazard low-risk based on the presence of right heart insufficiency and/or elevated cardiac biological markers.Therefore, in terms of treatment, for patients with acute stage non-high risk, the most important treatment is anticoagulant therapy. Rivaroxaban can be selected for initial treatment, and we used this drug for treatment of this patient.
diagnosis has preliminary results. What are the abnormal results of laboratory examinations, combined with relevant examinations, right pleural effusion examination, cardiac color ultrasound , and bi-pulmonary enhancement CT? How should we judge at this time?
Based on the above analysis, the team diagnosed the patient's admission as: 1. Bilateral pleural effusion test: infection? Pulmonary embolism? 2. Pulmonary embolism. Non-high-risk group; 3. Deep venous thrombosis in the right lower limb.
Further improve relevant examinations, the first is laboratory examination:
Arterial blood gas: PH7.46↑, PO2 105 mmHg, HCO3-28.4mmol/L, HCO3std 28.2mmol/L, SO2c 98%↑. Blood routine: hemoglobin 129g/L, eosinophil count 0.92×10^9/L↑, eosinophil ratio 12.30%, average erythrocyte hemoglobin concentration 315g/L, the rest is normal. Liver function: Total protein 64.9g/L, albumin 39.0g/L; myocardial enzyme : creatine kinase 33.9u/L; blood lipid : high-density lipoprotein cholesterol 1.00mmol/L; urinary routine, stool routine, renal function, electrolyte, fasting blood sugar , coagulation function, PCT, NT-proBNP, TnI have no abnormalities. D-dimer, ESR, CRP, no exception was found.
Tuberculosis Related: Tuberculosis complete set (blood draw): Tuberculosis Bacillus antibody (PPD-IgG) positive; PPD:1:2000 (+), 1:10000 (-). The Y interferon release test was negative.
connective tissue disease related: complement C3, complement C4, HCY, rheumatoid factor , ANA, C-ANCA, P-ANCA, a-ANCA, anti-cycloguanidine peptide antibody, anti-glomerular basement membrane antibody, anti-ENA antibody, autoimmune liver complete set, ENA, anti-cardiolipin antibody without any abnormalities.
Protein C and protein S are normal. Full set of parasites (-).
Right pleural effusion test: Routine: Li Fanta test was positive (+), total number of cells 32000×10^6/L, leukocyte 1350×10^6/L, mononuclear cells 0.65, multiple nuclear cells 0.35. Biochemistry: lactate dehydrogenase 241.1u/L: globulin 16.8g/L, adenosine deaminase 7.9u/L, total protein 49.0g/L, albumin 32.2g/L, glucose 7.22mmo1/L, chloride 106.7mmol/L. The complete set of tuberculosis: Budibacter tuberculosis antibody (PPD-IgG) is positive (weak), Budibacter tuberculosis antibody (PPD-IgM) is negative, and the marker test is negative; carcinoembryonic antigen is normal; acid-resistant staining (-); culture + drug sensitivity (-).
htmlOctober 29th cardiac color ultrasound: 1. Movie of the right atrium: The possibility of thrombosis is high; 2. Right pleural effusion (small amount). Color ultrasound of deep vein in both lower limbs: blood flow in deep veins in both lower limbs.
October 30th enhanced CT of both lungs (Figure 1) shows: double pneumonia and pleural effusion. Pulmonary artery CTA: 1. No obvious abnormalities were found in pulmonary artery CTA. 2. Multiple exudation lesions in both lungs and effusion in the right pleural cavity. (The patient started anticoagulation with rivaroxaban on September 13, and the pulmonary embolism has improved and disappeared).
Figure 1. In the treatment of lung enhancement CT
, oral rivaroxaban was reduced to 20 mg Qd, and the right pleural effusion was drained.
conducts a careful analysis of the causes of VTE and the causes of eosinophils. MDT consultation, can the answer that has surfaced this time be decided?
team is now starting to conduct a careful analysis of the causes of VTE and the causes of eosinophil eosinophil euphratica. The ideas are as follows:
, the causes of VTE. If VTE is repeated in patients <50>
Acquired thrombopathy includes: 1. Acquired thromboproliferative diseases: antiphospholipid syndrome, tumor diseases, myeloproliferative tumors, , paroxysmal sleep hemoglobinuria, , nephrotic syndrome, acute internal medicine diseases (congestive heart failure, severe respiratory diseases, etc.), inflammatory bowel disease, etc.; 2. Acquired thromboprone factors: surgery or trauma, long-term braking, advanced age, pregnancy and puerperal period, oral contraceptives and hormone replacement therapy, tumor treatment, acquired anticoagulant protein defects, etc.
However, the patient's examination did not find the cause of thrombophilia, but the patient's eosinophil is significantly increased. Can we find the cause from eosinophil growth as a breakthrough?
. Eosinophils are elevated. peripheral blood eosinophils absolute count >0.5×10^9/L can diagnose eosinophils. Hypereosinophilia is defined as: 2 peripheral blood tests (interval time > 1 month) eosinophil absolute count > 1.5×10^9/L and (or) bone marrow nucleated cell count eosinophil ratio 20% and (or) pathology confirms extensive infiltration of tissue eosinophils and (or) finds significant deposition of eosinophil granule protein (with or without more obvious tissue eosinophil infiltration).
This patient is in line with the diagnosis of eosinophilia, and its causes include hereditary, secondary (allergic diseases, skin diseases, drugs, infections, gastrointestinal diseases, vasculitis, rheumatism, respiratory diseases, tumors, etc.), clonality, idiopathicity and other major categories.
diagnostic procedures are as follows:
. When inquiring about medical history, you should carefully ask whether there are allergic diseases, whether there are rashes or lymph node enlargement, and whether there are cardiopulmonary and gastrointestinal symptoms. Are there any physical symptoms such as fever, night sweats, weight loss, itching and alcohol-induced pain? Inquiry in detail about the travel history, especially if there is a tropical region history;
. All patients with eosinophilia should undergo the following routine laboratory tests: complete blood count and peripheral blood smear classification count, routine biochemical examinations, including liver and renal function, electrolytes and lactate dehydrogenase. Red blood cell sedimentation rate and (or) C reactive protein , serum VitB12, etc.;
. Those asymptomatic and only mild to moderate eosinophilia [absolute eosinophil count (0.5-1.5) × 10^9/L] can not be further examined for the time being;
. If there are systemic symptoms or persistent eosinophilia (absolute eosinophil count ≥1.5 × 10^9/L) with or without suspicious organ damage, the following examination should be performed first to determine or rule out possible secondary causes.(1) Consider the causes of allergies: serum IgE, allergen-specific 1gE, skin acupuncture experiment for specific allergies; (2) Consider the non-allergic skin causes: skin biopsy; (3) Consider the causes of infection: stool parasites and worm egg microscopy, serological experiments for suspected infection of parasites, HIV and human T Cellular lymphophilic virus type I (HTLV-l); (4) Consider gastrointestinal causes: upper gastrointestinal endoscopy, small intestinal or anorectoscopy, serum amylase, and celiac disease-related autoantibodies; (5) Consider connective tissue disease: anti-nuclear antibodies (ANA) or anti-double-stranded NDA antibodies (dsDNA), citrulline cyclic peptide (ccp) antibodies; (6) Consider vasculitis: anti-neutrophil cytoplasmic antibodies (ANCA), serological tests of HBV, HCV, HIV, CMV and B19 viruses Measurement; (7) Consider respiratory diseases: imaging examination, branching microscopy;
5. Patients with eosinophilia without clear secondary causes and eosinophilia (absolute eosinophil count ≥1.5×10^9/L) should consider hematologic malignant tumors with clonal eosinophilia. In order to determine or rule out possible diseases, the following examinations should be conducted: bone marrow aspiration smear classification count, bone marrow biopsy, FISH or RT-PCR detection FIP1L1/PDGFRα, etc. For organ damage caused by suspected eosinophilia, the affected organ should be evaluated.
According to the above diagnosis and treatment ideas, among the common causes, the patient's parasite and connective tissue disease tests were all negative, and the patient had a rash. The next step is to further screen for skin biopsy, hematological system tumors, etc. to further clarify the diagnosis. Therefore, we invited MDT consultations in the Department of Hematology, Rheumatology and Immunology, Dermatology, Cardiology and Cardiology Surgery. The diagnosis after consultation is considered as: 1. Easymptomatic syndrome. Right atrial thrombosis. Bilateral pleural effusion; 2. Protein S deficiency? 3. Eosinophilic dermatosis? It is necessary to improve skin biopsy, and to detect ENA, ANA, platelet-derived growth factor genes and protein S genes in the dermatology laboratory. It is necessary to perform PET-CT to rule out early lymphoma. But the patient's parents refused PET-CT. Dermatology laboratory: ENA, ANA negative. Pathological results of skin biopsy in the rash: The epidermis and subcutaneous tissues were roughly normal, and sparse tissue cells were seen in the superficial vascular blood vessels of the dermis, and no eosinophils were seen.
After the blood internal medicine department's consultation again, it is recommended to improve the complete set of thrombosis, blood lipids, coagulation factor activity detection, protein S, protein C, HCY, anemia, and coagulation functions, and improve the sequencing of all exon genes if necessary. Later, the three items of blood lipids, HCY, anemia, protein C, and protein S were checked again without any abnormalities. Coagulation function: fibrinogen concentration 1.72g/L. Coagulation factor: ⅰ factor detection 300.2%↑ (50-100), fibrin degradation product 3.22ug/mL↑ (0-2.01). At the same time, complete the sequencing of all exon genes. Ethrombosis refers to a disease that causes various hereditary or acquired factors to be prone to thrombosis and thromboembolic. The patient's examination has not yet found a clear cause of hereditary thrombopathy, and it is currently considered to be a thrombopathy caused by acquired factors related to eosinophilia. The patient was finally discharged from the hospital on November 9 and was diagnosed as: 1. Estimation of thrombosis. Right atrial thrombosis. Bilateral pleural effusion; 2. Cause of rash: allergic dermatitis? Follow-up after discharge: Whole exon gene sequencing results: no mutations in thrombophilia-related genes were found. December 26, 2018: Qualitative test of peripheral blood FIP1L1/PDGFRα fusion gene: negative. Qualitative test of peripheral blood ETV6-PDGFRβ fusion gene: negative. Peripheral blood FISH test: negative. January 4, 2019: Cardiac color ultrasound: Pink clump-like echoes on European-style flap on the right atrium, which was significantly reduced compared with the previous one (2018.10.29). February 15, 2019: Cardiac color ultrasound: European-style valves are slightly thickened locally, tachycardia, and left ventricular systolic function measurement range. No pleural effusion was seen. (Table 2)
Table 2. Re-examination of eosinophils
After the patient was discharged from the hospital, the treatment was given rivaroxaban 20mg Qd, which was changed to 20mg Qod after February 2019; prednisone 15mg Qd was added in December 2018, and the dose was reduced to 10mg Qd in February 2019.
Just one wave was flat and the wave started again. The patient's arm showed a coin-sized lump, and the lymph nodes were enlarged? After the operation, the pathology revealed a rare disease, which was closely related to the previous eosinophil increase
I thought I would be calm after being discharged from the hospital, but unexpectedly, the wave just calmed down and then rose again. On May 6, 2019, the patient was admitted to the first ward of the orthopedic ward of our hospital for a painless mass on his right upper arm for 3 months. According to the patient's private complaint, starting from around February 2019, there was no obvious cause and found a painless lump in the right upper arm, about the size of a coin, and there was no significant enlargement after discovery and no tenderness.
The patient underwent a color ultrasound examination at Hunan Provincial Cancer Hospital in April of the same year and showed that there was a solid mass on the medial side of the right elbow. The outpatient clinic was admitted to the hospital with the nature of the right elbow lump to be examined. Since the onset of the disease, the patient's mental and appetite are still good, and the second stools are normal. During physical examination, it was found that a coin-sized lump could be paved on the inner side of the patient's right elbow, with no redness or swelling in the area, no tenderness, softness in texture, low mobility, and no obvious vasodilation in the area. There were no obvious abnormalities in cardiopulmonary and abdominal examinations. The diagnosis of admission was: 1. The nature of the right elbow mass is to be examined; 2. Easymptomatic syndrome.
At this time, the patient was subjected to a color ultrasound of the body surface: the upper acoustic image of the bilateral elbow joint, and the lymph node enlargement was considered. Heart color ultrasound: No obvious abnormalities were found in the intracardiac structure. No significant abnormalities were found in lung CT. So on May 10, 2019, the patient was subjected to a right elbow mass removal surgery (Figure 2).
Figure 2. Right elbow lymph node pathology
Results, postoperative pathology showed that it was consistent with Kimura disease (Kimura disease). The final diagnosis was: 1. Kimura disease ; 2. Easymptomatic disease.
Kimura’s disease, also known as eosinophilic hyperplastic lymphoma, is a rare chronic progressive inflammatory disease with unknown cause and mainly damage to lymph nodes, soft tissues and salivary glands. It is popular among East Asians, middle-aged and young men. Its clinical characteristics are: multiple (seldom single) masses of soft tissue are the most common clinical manifestations, mostly located in the subcutaneous soft tissue in the head and neck area. Common areas: peri-ear, groin, scalp, periorbital, eyelid, etc.; lump characteristics: painless, unclear boundaries, adhesions to surrounding tissues, poor mobility; local lymph nodes, large salivary glands such as parotid glands, submandibular glands are often involved and enlarged; skin itching and pigmentation incidence is 40%-100%, and skin on the lump is often present; 12%-16% of patients are accompanied by proteinuria.
Laboratory examination: The proportion and count of eosinophils in peripheral blood increased significantly, serum IgE levels increased significantly, and bone marrow aspiration found that eosinophils in the bone marrow also increased significantly, mainly in the late childhood and mature stages; imaging examinations were not specific. Pathology: The hyperplasia and infiltration of inflammatory cells , including extensive lymphoid follicular hyperplasia in the lesion tissues and a large number of eosinophils, lymphocytes, and mast cells that flood between the follicles; eosinophils accumulate focally in the lymph nodes to form eosinophils microabscesses, invading germinal center can cause necrosis and structural damage to the germinal center cells, which is called eosinophilic follicle lysis; capillaries proliferate a large amount of capillaries, swelling and significantly proliferating vascular endothelial cells, causing thickening of the tube wall and even obstruction of the lumen; the soft tissue lesions are obvious, and fibrous tissue with varying degrees of hyperplasia wraps around and separates the lesion tissue.
Diagnosis: There is no unified diagnostic standard in the world. According to the typical clinical manifestations of the good-haired population in the good-haired area, the painless lump slowly occurs on the head and neck of middle-aged and young Asian men, there are no other signs of infection, and the boundaries of the lumps are unclear. If there is or is itching, accompanied by or not with lymph nodes and large salivary gland enlargement, increased absolute value and proportion of peripheral blood eosinophils and significantly increased serum IgE levels, the possibility of this disease should be considered. Pathological biopsy shows eosinophil infiltration in lymph nodes, focal aggregation to form microabscesses, and can be confirmed by different degrees of fibrosis and vascular hyperplasia.
Differential diagnosis: Angiolymphoid hyperplasia with eosinophilia (ALHE): ALHE is found in all races, mainly in white people, with little gender difference, rash and subcutaneous nodules are the main lesions; pathological examination of lymph node hyperplasia but no follicular formation, poor small blood vessel formation, and atypical endothelial cells are accompanied by lymphocytes and eosinophil infiltration; no renal damage. The two are different in terms of vascular lesions. The vascular changes in Kimura disease are mainly due to the increase in the number of endothelial cells in the thin-walled blood vessels, which are lighter than ALHE, while ALHE involves thick-walled blood vessels, forming histocyte-like or epithelial-like endothelial cells, which may be accompanied by cytoplasmic vacuolation, and excessively proliferated endothelial cells protrude into the lumen in the shape of fingers, and even causing vascular obstruction.
Treatment aspect: Surgery: single-onset, small lumps, easy to remove lesions; radiotherapy: large range of lesions, multiple occurrences, unclear boundaries or local infiltration, recurrence after surgery; glucocorticoids and cyclosporine. Prognosis: benign course, no tendency to change malignantly, good prognosis, easy to recur.
Although the elbow mass in this case is not a typical site for Kimura's disease, it can be diagnosed as Kimura's disease based on the region, age, gender, increased eosinophils and IgE, and pathology of the disease. At this point, the diagnosis of this case has been revealed, but the experience and experience given to us are: 1. It reminds us that for young patients with pulmonary embolism without obvious causes, we need to further investigate the cause of pulmonary embolism and whether there is thromboproliferative syndrome; 2. For patients with pulmonary embolism with eosinophilic eruption, eosinophilic eruption can be used as a breakthrough in finding the cause.
References
[1] Sun Xuefeng, Liu Tao, Cai Baiqiang. Interpretation of the 2010 Guidelines for Diagnosis of Unilateral Pleucorrhoeae in Adults by the British Thoracic Society. International Journal of Respiratory, 2011, 31(10): 721-725.
[2] Pulmonary embolism and pulmonary vascular disease group of the Chinese Medical Association, Pulmonary embolism and pulmonary vascular disease working committee of the Chinese Medical Association Respiratory Physicians Branch, and National Pulmonary Embolism and pulmonary vascular disease prevention and control cooperation group. Guidelines for diagnosis, treatment and prevention of pulmonary thromboembolic disease. Chinese Journal of Medicine, 2018, 98(14): 1060-1087.
[3] Thrombosis and hemostasis group of the Hematology Branch of the Chinese Medical Association. Chinese Expert Consensus on Diagnosis of Easythrombosis (2012 Edition). Chinese Journal of Hematology. 2012, (11): 982.
[4] Leukemia Lymphoma Group, Hematology Branch of the Chinese Medical Association. Chinese Expert Consensus on Diagnosis and Treatment of Eosinophilia (2017 Edition). Chinese Journal of Hematology, 2017, 38(7): 561-565.
[5] Cheng Maojie, Chang Jianmin. Kimura disease. Chinese Journal of Dermatology, 2010, 43(3), 218-220.
[6] Abuel-Haija M, Hurford M T. Kimura disease. Kimura disease. Archives of pathology & laboratory medicine, 2007, 131(4): 650-651.
Expert introduction
Luo Hong
Professor, first-level chief physician, doctoral supervisor, Xiangya Second Hospital of Central South University Director of the Department of Inhalation and Critical Care Medicine, Director of the Critical Sub-specialty, Member of the Respiratory Critical Care Group of the Respiratory Critical Care Branch of the Chinese Medical Association, Member of the Internal Medicine Branch of the Chinese Medical Association, Member of the Respiratory Physicians Branch of the Chinese Medical Association, Deputy Chairman of the Critical Care Medicine Working Committee of the Respiratory Physicians Branch of the Chinese Medical Association, Member of the Standing Committee of the Pulmonary Rehabilitation Committee of the Chinese Disabled Rehabilitation Association and Deputy Head of the ICU Pulmonary Rehabilitation Group, President of the Respiratory Physicians Branch of the Hunan Medical Association, and Deputy Chairman of the Respiratory Physicians Professional Committee of the Hunan Medical Association.
Ouyang Ruoyun
Chief physician, professor, doctor of medicine, doctoral supervisor of Xiangya Second Hospital, Central South University. In 2012, he went to the University of Southern California Medical Center to study for one year. Research direction: Sleep breathing disorders, rare lung diseases.He serves as a member of the Sleep Breathing Disorders Group of the 11th Committee of the Respiratory Medicine Branch of the Chinese Medical Association, a member of the Sleep Breathing Disorders Working Committee of the Respiratory Medicine Branch of the Chinese Medical Association, a director of the Rare Disease Branch of the China First Research Hospital Society, and a member of the First Standing Committee of the Respiratory Medicine of the China Rare Disease Alliance. He presided over 1 national-level project, participated in 3 national-level projects, presided over 4 provincial-level projects, won 3 provincial-level achievement awards, and published more than 60 professional academic papers, including more than 10 SCI papers, more than 10 Medline papers, and 2 hospital medical new technology awards.
Li Jinhua
Attendant physician of the Department of Respiratory and Critical Care Medicine, Xiangya Second Hospital, Central South University, Ph.D. Member of the Youth Committee of the Respiratory Medicine Professional Committee of Hunan Medical Association, presided over a project of the National Natural Science Foundation and a project of the Hunan Natural Science Foundation. Main research direction: respiratory critical illness.
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Editor: Dong Xuening; Editor: Jerry
