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A rare case of M3 found in an unhealed over-the-wall fracture

 Last Update: 2022-08-12
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foreword

Acute promyelocytic leukemia (M3) is a specific type of acute myeloid leukemia (AML), accounting for 10% of childhood myeloid leukemi.

blood vessels in children with leukemia

case after

A 10-year-old male patient fell accidentally while climbing a 3-meter-high wall 5 days a.

Diagnosis :Right pubic fracture and right iliac medial anterior border avulsion fracture;Left frontal scalp hematoma;Disseminated intravascular coagulation? During the hospitalization, the patient was immobilized to promote fracture healing, pain relief, blood transfusion (4 units of cryoprecipitate) and other treatmen.

diagnosis

External hospital examination: WBC 92×109/L, HGB 72g/L, PLT 43×109/L, NEUT 64%, LYMPH 3

Other examinations: Cranial CT in our hospital showedHematoma on the left forehead scalp, and the rest of the cranial CT showed no abnormali.

【Laboratory Inspection】 :

【Laboratory examination】

Blood routine: WBC 3×109/L, RBC 58×1012/L, HGB 69 g/L, PLT 42×109/L, see Figure

Figure 1 Blood routine report

The five results of coagulation are shown in Figure 2:

Figure 2 Report of 5 items of coagulation

Partial biochemical results: LDH: 3594 U/L↑ (120-250); CRP: 663 mg/L? (0-10); FERR: 8473ng/L (20-200) ↑

After admission, the children were monitored for hemoglobin, platelets, and coagulation functions, and a large number of cryoprecipitate, plasma, and suspended red blood cells were repeatedly infused, but the coagulation function was difficult to correct, and the fibrinogen decreased repeated.

*The first peripheral blood smear examination showed abnormal promyelocytes Figure 3, Figure 4:

Figure 3 Wright staining of peripheral blood×*10

Figure 4 Wright staining of peripheral blood × 1000

*Bone marrow smears are shown in Figures 5 and 6:

Figure 5 Wright staining of bone marrow × 1000

Figure 6 Bone marrow MP0 staining×1000

Morphological results of bone marrow cells: bone marrow smear showed a large number of abnormal promyelocytes with irregular karyotype, increased granules, and obvious internal and external plasma, the proportion was as high as 89%, and MPO staining was strongly positi.

According to the bone marrow morphology report, the doctor promptly treated the child with all-trans retinoic ac.

Figure 7 Wright staining of peripheral blood after treatment × 1000

Figure 8 Wright staining of peripheral blood after treatment × 1000

Flow cytometry results on July 14, consistent with the APL immunophenotype , see Figure 9:

immunity

Figure 9 Flow cytometry results

On July 14, the 5 fusion gene screening report showed that WT1 was overexpressed and the PML-RARa fusion gene was negative, as shown in Figure 10:

screening

Figure 10 56 fusion gene screening report

The karyotype analysis results submitted for inspection on July 14 were 47, XY, +21[8]/46, XY[2], see Figure 11:

Figure 11 Chromosome results

The flow cytometry results on August 22 showed no obvious abnormal immunophenotype of promyelocytes, as shown in Figure 12:

Figure 12 Flow cytometry results

On August 22, the bone marrow chromosome karyotype analysis results, 46, XY[16], are shown in Figure 13:

Figure 13 Bone marrow chromosome results

Changes in coagulation function analysis results after treatment are shown in Figure 14:

Figure 14 Changes in coagulation analysis results after treatment

case analysis

The morphology of the bone marrow cells of this patient is consistent with M3, and the flow cytometry results are consistent with the immunophenotype of acute promyelocytic leukemia (AP.

On July 14, the karyotype analysis of bone marrow cells showed karyotype: 47, XY, +21[8]/46, XY[2] (interpretation of the results: analysis of 10 metaphases showed that 8 had trisomy 21, abnormal ratio of 80.

On August 12, the karyotype of peripheral blood was analyzed, and the G-banding analysis of cell culture method showed that the karyotype was 46, XY, add(15)(p1

guide

This patient started treatment with retinoic acid on July 1 Through retrospective analysis of peripheral blood cell morphology and coagulation function from July 14 to July 25, differentiation syndrome occurred after retinoic acid induction therapy, and peripheral blood smear The proportion of abnormal promyelocytes gradually decreased, the proportion of middle and late myelocytes and mature granulocytes increased, and the cell morphology gradually developed to the normal direction, and the coagulation function was also improv.

Considering that the child is a low-income household, poor economic conditions, and his father is in his 60s, his health awareness is not stro.

Infect

Summarize

Acute promyelocytic leukemia (M3) is a leukemia with specific karyotype and genetic alterations, typical t(15,17) is present in 90% of patients, and PML-RARa fusion gene can be detected in 99% of patients [

It has been reported in the literature that AML with negative PML/RARa fusion gene morphologically consistent with APL is rare in clinical practi.

When there is no condition to detect ATRA, ATO sensitive genes or the two drugs have poor efficacy, induction therapy of acute myeloid leukemia chemotherapy should be applied as soon as possible to increase the remission rate and prognosis of patients [

There are few reports of APL with acquired trisomy 2 In China, Lu Ying et .

[4] counted 31 of 3329 cases of newly diagnosed AML patients with acquired trisomy 21, and none of them were M3 patients, which is very ra.

statistics

According to the domestic literature, there is no M3 report of acquired trisomy 21 and no t(15,17) by routine chromosomal detection [
However, the PML/RARa fusion gene negative and acquired trisomy 21 with morphological conformity to APL have not been report.

Therefore, the incidence rate is low, and there is no large-scale data statistics on the incidence and prognosis of such patien.

In this case, the bone marrow chromosome of this patient was 47, XY, +21[8]/46, XY[2] at the initial stage of treatment, and the karyotype analysis result of peripheral blood during the treatment was 46, XY, add(15)(p10), and the disease treatment remission After re-examination of bone marrow chromosomes showed a normal karyotype, indicating that acquired trisomy 21 may have a certain relationship with the pathogenesis of M Although the patient with PML/RARa fusion gene was negative and acquired trisomy 21, fortunately, the oral treatment of retinoic acid (ATRA) and compound Huangdai tablet was effective, but unfortunately no NGS test was performed, otherwise more RARa gene could be found partner gen.

【references】

[1] NCCNClinical Practice Guidelines in Oncology (NCCN Guidelines.

Acutemyeloid leukem.

Version 2020

[2] Chinese Medical Association Hematology Branch, Chinese Medical Doctor Association Hematologist Bran.

Guidelines for the diagnosis and treatment of acute promyelocytic leukemia in China (2014 edition) [.

Chinese Journal of Hematolo.

2014, 35 (5); 475-477

[3] Su Yutai, Liu Wei, Guan Yujie, Song Lili, Xie Xinshe.

Clinical observation of PML/RARa gene-negative acute myeloid leukemia morphologically consistent with APL [.

China Journal of Modern Medicine, 2019, 21(06) : 42-4

[4] Lu Ying, Yuan Jiaojiao, Wang Huafeng, e.

Clinical features and prognosis of acquired trisomy 21 acute myeloid leukemia [.

Chinese Journal of Medical Genetics, 2017, 34(4): 554-558

[5] Ying Shuangwei, Zhu Xiayin, Shen Jian, Luo Wenda, Zhang .

A case of acute promyelocytic leukemia with acquired trisomy 21 as the only cytogenetic abnormality [.

Modern Practical Medicine, 2019, 31 (2 ), 274-27

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