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Main content summary: Hepatosplenomegaly is a common clinical manifestation of patients with myelofibrosis (MF).
For patients with better coagulation function, conventional anticoagulation therapy with aspirin may not be used.
It is recommended that patients with doubtful diagnosis undergo a comprehensive examination to further confirm the diagnosis.
Professor Zhang Min of Wuhan Union Medical College Hospital, Professor Liu Zhenfang of the First Affiliated Hospital of Guangxi Medical University, Director Tang Xiaoyu of Beihai People's Hospital, and Doctor Li Lianqiao of Hainan Cancer Hospital, etc.
exchanged and introduced their experience in the field of MPN diagnosis and treatment through live online conferences.
I hope that through the Q&A and discussion of cases in this meeting, everyone can gain something.
Part1——Polycythemia vera secondary myelofibrosis case sharing, director Tang Xiaoyu of Beihai People's Hospital first shared a case of polycythemia vera secondary myelofibrosis (Post-PV MF).
Case sharing Case profile Patient Deng, female, 64 years old.
In November 2016, she went to the doctor due to "abdominal distension".
Three-line hyperplasia of granular red giant, increased number of red blood cells.
Genetic test: JAK2V617F mutation is positive.
Diagnosis: Polycythemia vera (PV) combined with MF, treated with hydroxyurea.
In February 2020, the patient was admitted to the hospital again due to "progressive abdominal distension, fatigue and mobility impairment".
Physical examination: poor spirit, weight loss, anemic appearance, bulging abdomen, 20cm below the ribs of the spleen.
Auxiliary blood test: HGB: 57g/L Biochemistry: LDH: 2486U/L Abdominal color Doppler ultrasound: liver size and shape is normal, spleen color super thick meridian: 110mm, oblique meridian 281mm Gene detection: JAK2V617F mutation positive case diagnosis Post-PV MF (IPSS: 2 points, DIPSS: 3 points, intermediate risk-2) After diagnosis and treatment, thalidomide 50 mg qd, stanozolol 2 mg bid, blood transfusion, diuresis, and infection control after 2020-02.
The patient's fatigue symptoms improved.
2020-09 The patient was again admitted to the hospital due to fatigue and abdominal distension, accompanied by night sweats, itching, bone pain, and progressive weight loss.
Physical examination: poor spirit, anemia, weight loss.
Blood routine: HGB: 70g/L, MPN-10 score: 52 points.
Rucotinib 15mg bid was given.
The follow-up visit on 2020-09 showed that the patient's blood routine indicators did not decrease, hemoglobin was stable, fatigue improved, skin itching, bone pain disappeared, abdominal distension and appetite were significantly improved.
The 2020-10 patient was hospitalized again due to edema, abdominal distension, and asthma.
Physical examination: appearance of anemia, edema of limbs, and a large amount of fluid in the abdominal cavity, which increased compared to the previous period.
The blood routine index was stable, and he was treated with a tube in the abdominal cavity and diuretic treatment, and his condition improved and was discharged from the hospital.
During the period, rucotinib was not discontinued.
Because the patient's blood test showed that the indicators were stable, the amount of rucotinib was increased to 20 mg bid.
The MPN-10 score of the patient's latest follow-up visit: 13 points.
Blood routine: HGB: 84g/L, WBC and PLT are normal.
How to explain the hepatomegaly that appears in the course of the patient's medication? The guidelines recommend the routine use of aspirin for PV anticoagulation, but based on the patient's current condition, does the patient need conventional anticoagulation therapy? What is the cause of ascites during the course of the patient's illness? What are the recommendations for later treatment (whether to continue the combination medication and possible benefits) and how to estimate the possible prognosis of the later disease? Professor Zhang Min’s comments: The patient’s medical history before 2016 is lacking, so the diagnosis is controversial, and it is impossible to determine whether it is Post-PV MF or primary myelofibrosis (PMF).
It is recommended that the patient undergo a peripheral blood smear and FISH test to improve the diagnosis.
Hepatosplenomegaly is a common clinical manifestation of MF patients.
Patients with MF often have extramedullary hematopoiesis in the liver and spleen, leading to hepatosplenomegaly and portal hypertension.
Symptomatic treatment is recommended.
If the patient's coagulation function is good, conventional anticoagulation therapy with aspirin may not be used.
Part2-Suspected Acute Myeloid Leukemia Secondary Myelofibrosis Case Sharing Dr.
Li Lianqiao from Hainan Cancer Hospital then shared a suspected acute myeloid leukemia (AML) secondary MF case.
Case sharing Case profile The patient is male, 53 years old.
Due to "weakness for 5 months", he was admitted to the hospital in June 2019 and was diagnosed with AML (chromosome +8) with a medium prognosis group.
He was treated with decitabine, idarubicin, and cytarabine.
He was admitted to the hospital again in July 2020 due to "abdominal bloating for 1 month".
Auxiliary examination physical examination: no appearance of anemia, no bleeding spots and ecchymosis on the skin and mucous membranes, no tenderness in the sternum, under the liver and ribs, 10 cm under the spleen and ribs, routine hard blood: WBC: 20.
77×109/L, HGB: 98g /L, PLT: 62×109/LAML MRD test: no leukemia residual cells AML fusion gene: negative peripheral blood MPN-related gene mutation and fusion test: ASXL1 (mutation frequency 42.
5%), RUNX1 (mutation frequency 43.
2%), SRSF2 (Mutation frequency 45.
6%), EZH2 (Mutation frequency 43.
6%) bone marrow biopsy: Bone marrow hyperplasia is more active, granular red giant tri-lineage cell hyperplasia, fibrosis can be seen, no obvious increase in blasts.
HE and PAS staining showed that bone marrow hyperplasia was more active (>90%), the number of megakaryocytes was roughly normal, and megakaryocytes with small cell bodies were seen.
Focal hyperplasia of fibrous tissue.
Reticular fiber staining (grade MF-3) diagnoses AML secondary to MF? PMF during blast? After diagnosis and treatment, azacitidine 100mg×7d q28d combined with rucotinib 5mg bid was given on 2020-07-26.
2020-08-26 The patient's platelet level rebounded, and Rucotinib was increased to 10mg big.
On September 23, 2020, the patient's platelet level further rebounded, and rucotinib was increased to 15 mg bid.
The curative effect judges that the patient has a better effect of shrinking the spleen, and the blood picture quickly returns to the normal level.
Professor Zhang Min’s comments: It is recommended that the patient undergo a comprehensive examination to further confirm the diagnosis.
This patient tends to AML with MF.
The MCC number JAK21032450 is valid for 2022-03-17, and the information is expired and deemed invalid.
This information is only for personal academic reference by medical and health professionals.
Poke "read the original text" and we will make progress together
For patients with better coagulation function, conventional anticoagulation therapy with aspirin may not be used.
It is recommended that patients with doubtful diagnosis undergo a comprehensive examination to further confirm the diagnosis.
Professor Zhang Min of Wuhan Union Medical College Hospital, Professor Liu Zhenfang of the First Affiliated Hospital of Guangxi Medical University, Director Tang Xiaoyu of Beihai People's Hospital, and Doctor Li Lianqiao of Hainan Cancer Hospital, etc.
exchanged and introduced their experience in the field of MPN diagnosis and treatment through live online conferences.
I hope that through the Q&A and discussion of cases in this meeting, everyone can gain something.
Part1——Polycythemia vera secondary myelofibrosis case sharing, director Tang Xiaoyu of Beihai People's Hospital first shared a case of polycythemia vera secondary myelofibrosis (Post-PV MF).
Case sharing Case profile Patient Deng, female, 64 years old.
In November 2016, she went to the doctor due to "abdominal distension".
Three-line hyperplasia of granular red giant, increased number of red blood cells.
Genetic test: JAK2V617F mutation is positive.
Diagnosis: Polycythemia vera (PV) combined with MF, treated with hydroxyurea.
In February 2020, the patient was admitted to the hospital again due to "progressive abdominal distension, fatigue and mobility impairment".
Physical examination: poor spirit, weight loss, anemic appearance, bulging abdomen, 20cm below the ribs of the spleen.
Auxiliary blood test: HGB: 57g/L Biochemistry: LDH: 2486U/L Abdominal color Doppler ultrasound: liver size and shape is normal, spleen color super thick meridian: 110mm, oblique meridian 281mm Gene detection: JAK2V617F mutation positive case diagnosis Post-PV MF (IPSS: 2 points, DIPSS: 3 points, intermediate risk-2) After diagnosis and treatment, thalidomide 50 mg qd, stanozolol 2 mg bid, blood transfusion, diuresis, and infection control after 2020-02.
The patient's fatigue symptoms improved.
2020-09 The patient was again admitted to the hospital due to fatigue and abdominal distension, accompanied by night sweats, itching, bone pain, and progressive weight loss.
Physical examination: poor spirit, anemia, weight loss.
Blood routine: HGB: 70g/L, MPN-10 score: 52 points.
Rucotinib 15mg bid was given.
The follow-up visit on 2020-09 showed that the patient's blood routine indicators did not decrease, hemoglobin was stable, fatigue improved, skin itching, bone pain disappeared, abdominal distension and appetite were significantly improved.
The 2020-10 patient was hospitalized again due to edema, abdominal distension, and asthma.
Physical examination: appearance of anemia, edema of limbs, and a large amount of fluid in the abdominal cavity, which increased compared to the previous period.
The blood routine index was stable, and he was treated with a tube in the abdominal cavity and diuretic treatment, and his condition improved and was discharged from the hospital.
During the period, rucotinib was not discontinued.
Because the patient's blood test showed that the indicators were stable, the amount of rucotinib was increased to 20 mg bid.
The MPN-10 score of the patient's latest follow-up visit: 13 points.
Blood routine: HGB: 84g/L, WBC and PLT are normal.
How to explain the hepatomegaly that appears in the course of the patient's medication? The guidelines recommend the routine use of aspirin for PV anticoagulation, but based on the patient's current condition, does the patient need conventional anticoagulation therapy? What is the cause of ascites during the course of the patient's illness? What are the recommendations for later treatment (whether to continue the combination medication and possible benefits) and how to estimate the possible prognosis of the later disease? Professor Zhang Min’s comments: The patient’s medical history before 2016 is lacking, so the diagnosis is controversial, and it is impossible to determine whether it is Post-PV MF or primary myelofibrosis (PMF).
It is recommended that the patient undergo a peripheral blood smear and FISH test to improve the diagnosis.
Hepatosplenomegaly is a common clinical manifestation of MF patients.
Patients with MF often have extramedullary hematopoiesis in the liver and spleen, leading to hepatosplenomegaly and portal hypertension.
Symptomatic treatment is recommended.
If the patient's coagulation function is good, conventional anticoagulation therapy with aspirin may not be used.
Part2-Suspected Acute Myeloid Leukemia Secondary Myelofibrosis Case Sharing Dr.
Li Lianqiao from Hainan Cancer Hospital then shared a suspected acute myeloid leukemia (AML) secondary MF case.
Case sharing Case profile The patient is male, 53 years old.
Due to "weakness for 5 months", he was admitted to the hospital in June 2019 and was diagnosed with AML (chromosome +8) with a medium prognosis group.
He was treated with decitabine, idarubicin, and cytarabine.
He was admitted to the hospital again in July 2020 due to "abdominal bloating for 1 month".
Auxiliary examination physical examination: no appearance of anemia, no bleeding spots and ecchymosis on the skin and mucous membranes, no tenderness in the sternum, under the liver and ribs, 10 cm under the spleen and ribs, routine hard blood: WBC: 20.
77×109/L, HGB: 98g /L, PLT: 62×109/LAML MRD test: no leukemia residual cells AML fusion gene: negative peripheral blood MPN-related gene mutation and fusion test: ASXL1 (mutation frequency 42.
5%), RUNX1 (mutation frequency 43.
2%), SRSF2 (Mutation frequency 45.
6%), EZH2 (Mutation frequency 43.
6%) bone marrow biopsy: Bone marrow hyperplasia is more active, granular red giant tri-lineage cell hyperplasia, fibrosis can be seen, no obvious increase in blasts.
HE and PAS staining showed that bone marrow hyperplasia was more active (>90%), the number of megakaryocytes was roughly normal, and megakaryocytes with small cell bodies were seen.
Focal hyperplasia of fibrous tissue.
Reticular fiber staining (grade MF-3) diagnoses AML secondary to MF? PMF during blast? After diagnosis and treatment, azacitidine 100mg×7d q28d combined with rucotinib 5mg bid was given on 2020-07-26.
2020-08-26 The patient's platelet level rebounded, and Rucotinib was increased to 10mg big.
On September 23, 2020, the patient's platelet level further rebounded, and rucotinib was increased to 15 mg bid.
The curative effect judges that the patient has a better effect of shrinking the spleen, and the blood picture quickly returns to the normal level.
Professor Zhang Min’s comments: It is recommended that the patient undergo a comprehensive examination to further confirm the diagnosis.
This patient tends to AML with MF.
The MCC number JAK21032450 is valid for 2022-03-17, and the information is expired and deemed invalid.
This information is only for personal academic reference by medical and health professionals.
Poke "read the original text" and we will make progress together
