Thrombotic thrombocytopenic purpura complicated with acute aortic dissection: A case report.

RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a critical thrombotic microangiopathy involving multiple organs. To the best of our knowledge, there are no reports of TTP complicated by acute aortic dissection. PATIENT CONCERNS: We herein described a 53-year-old male with TTP who did not have a significant medical history. After immediate plasma exchange and glucocorticoid therapy, the patient's clinical condition improved. However, the patient suddenly experienced chest pain with elevated blood pressure. DIAGNOSES: Computed tomography angiography suggested acute type B aortic dissection. INTERVENTIONS: The patient was immediately transferred to the cardiac aortic surgery department for thoracic aortic endovascular repair. OUTCOMES: The patient was discharged after successful thoracic aortic endovascular repair. Unfortunately, 3 months later, the patient experienced chest and back pain at home and died suddenly, possibly due to the recurrence of aortic dissection. LESSONS: Even if patients have no identifiable risk factors, physicians should be aware of this rare and life-threatening acute complication of TTP, which may have multiple causes, including preexisting connective tissue disease, abnormal blood pressure fluctuations, and increased risk of hemorrhage. Early identification and timely treatment of acute aortic dissection are critical for improving prognosis.

[CD96 expression on bone marrow mononuclear cells in 91 patients with acute leukemia].

This study was aimed to investigate the expression of CD96 on bone marrow mononuclear cells (BMMNC) from 91 patients with acute leukemia, and the results were analyzed with clinical pathological data. Flow cytometry was used to detect CD96 molecule on the bone marrow mononuclear cell surface of 91 newly diagnosed patients with acute leukemia, and 15 healthy adults were served as normal controls. The results showed that the average rate of CD96(+) expression on BMMNC (CD45(+) CD34(+) CD19(+)) of 21 patients with B-ALL was (17.41 ± 27.97)%, the average rate of CD96(+) expression on stem cells (CD45(+)CD34(+)CD7(+)) of 11 patients with T-ALL was (46.98 ± 45.55)%, the average rate of CD96(+) expression on BMMNC (CD45(+)CD34(+)CD38(-)) of 59 patients with AML was (16.69 ± 25.08)%, while the average rate of CD96(+) on BMMNC of healthy adult controls was (0.52 ± 1.84)%, there was significant difference in average rate of CD96(+) expression between above-mentioned patients and healthy adult controls (p < 0.05). Otherwise the average rate of CD96(+) on BMMNC after treatment showed no statistical difference between patient group with CR (1.68 ± 2.31) and healthy controls, but demonstrated statistical difference between patients without CR and healthy controls (p > 0.05). The leukocyte count, hemoglobin level and platelet count in CD96(+) group had no obvious difference from CD96(-) ones (p > 0.05). No change found in the field of molecular biology and cytogenetic between these 2 groups. It is concluded that CD96 expression is different in different types of leukemia. The positive expression of CD96 on bone marrow hematopoietic stem cells in patients with acute leukemia may be associated with primary drug resistance, relapse and progression. The CD96 on BMMNC of acute leukemias can be a helpful prognostic indicator in treatment response assessment.

[Diagnostic role of BIOMED-2 multiplex polymerase chain reaction for antigen receptor genes rearrangement in lymphoproliferative disorders].

OBJECTIVE: To establish a sensitive and effective method for detection of immunoglobulin and T-cell receptor (Ig/TCR) gene rearrangement,and to explore its role in diagnosis and differential diagnosis of lymphoproliferative disorders. METHODS: Fifty-eight lymphoid tissue samples from 54 patients with lymphoproliferations were evaluated by the novel BIOMED-2 multiplex polymerase chain reaction (PCR) for antigen receptor genes rearrangement. RESULTS: Multiplex PCR demonstrated monoclonal Ig/TCR gene rearrangements in 22 of 25 (88.0%) B-cell malignancies and 8 of 15 (53.3%) T-cell malignancies. Among 17 benign lymphoproliferations confirmed histopathologically, polyclonal rearrangements were detected in 14 cases (82.4%). In total, the clonality analysis and the final clinico-histopathological diagnosis were concordant in 77.2%. Combination detection of Iglambda and TCR delta gene rearrangements did not increase the detection rate of monoclonal rearrangement of Ig/TCR, but might help to the detection of Iglambda+ or TCR delta+ lymphomas. CONCLUSION: The novel BIOMED-2 multiplex PCR strategy is a rapid, reliable and sensitive approach to detecting clonality in suspected lymphoproliferations, especially in atypical cases.

[Clinical analysis of amphotericin B in the treatment of invasive fungal infections in 121 patients with hematologic diseases].

OBJECTIVE: To observe the efficacy and safety of amphotericin B for treatment of invasive fungal infections (IFI) in patients with hematologic diseases. METHODS: 121 patients were given amphotericin B 5 -50 mg/d for 5 - 101 d with a median of 19 d. RESULTS: The clinical efficacy rate was 67.3%, and fungal elimination rate 66.7%. The adverse events included rigor and fever, hypokalaemia, hepatic damage, nephrotoxicity, nausea and vomiting, phlebitis and teeter. CONCLUSION: Amphotericin B is still a high-efficiency drug in the treatment of IFI, although it has many side effects. With monitoring of hepatic and renal function, it is still a relatively safe and effective drug.

[ERK pathway change in the differentiation of human MDS cell lines SKM-1 induced by sodium butyrate].

The study was purposed to investigate the role of extracellular signal-regulated kinase (ERK) pathway in the differentiation of human MDS cell lines SKM-1 induced by sodium butyrate (NaB), and to elucidate the molecular mechanism of differentiation in SKM-1 cells induced by NaB. The expression levels of total ERK and phosphorylated-ERK were determined by Western blot. The effect of NaB in combination with the ERK inhibitor PD98059 on the proliferation/differentiation of SKM-1 cells was studied, and then the expression levels of the P21 and HDAC protein were detected by Western blot. The results showed that the expression level of phosphorylated ERK was down-regulated by the 1 mmol/L NaB, and the level of total ERK had not changed. NaB or combination of the MEK inhibitor PD98059 with NaB could increase the differentiation of the SKM-1 cells and up-regulated the levels of the P21 and HDAC protein, but the effect of combination of NaB with PD98059 was higher than that of NaB alone. It is concluded that the inhibition of ERK may be involved in sodium butyrate inducing differentiation in SKM-1 cells.

[The clinical application of splenectomy in pyrexia of unknown origin with splenomegaly].

OBJECTIVE: To observe the clinical value of splenectomy for pathologic diagnosis in fever of unknown origin with splenomegaly only. METHODS: The pathologic findings of 35 patients with fever of unknown origin and splenomegaly treated by splenectomy, admitted in to the department of hematology in our hospital since 1996 were studied retrospectively. For these patients, there were no other positive signs except splenomegaly and the routine tests could not help us make the etiological diagnoses. RESULTS: In these 35 patients, there were 17 cases of non-Hodgkin's lymphoma (48.6%), 5 cases of Hodgkin's disease (14.2%), 2 cases of malignant histiocytosis (5.7%), 5 cases of connective tissue disease (14.2%), 2 cases of chronic congestive splenomegaly (5.7%), 1 case of hemophagocytic syndrome (2.9%), 1 case of remote spleen infarction (2.9%), 1 case of tuberculosis of spleen (2.9%) and 1 case of spleen angiosarcoma (2.9%). CONCLUSION: When only splenomegaly is found in patients with fever of unknown origin, it is necessary to persuade the patients to accept diagnostic splenectomy for pathological as soon as possible, otherwise, the diagnosis and treatment may be delayed.

[Effect of sodium butyrate in combination with ATRA on the proliferation/differentiation of MDS cell line SKM-1].

The study was purposed to explore the molecular mechanisms of sodium butyrate (NaB) action on SKM-1 cell proliferation/differentiation and to study its synergistic effect with all-trans retinoic acid (ATRA). SKM-1 cells were grown in the absence or presence of NaB and/or ATRA; the percentage of viable cells was determined by trypan blue exclusion; differentiation was investigated by nitro-blue tetrazolium (NBT) reduction; adhesion molecules of cell surface were analysed by FACS; cell cycle distribution was studied after DNA staining by propidium iodide; D-type cyclins, CDK and P21 mRNA were detected by reverse transcription-polymerase chain reaction (RT-PCR). The results showed that NaB and/or ATRA blocked cells mainly in the G0/G1 phase of the cell cycle; ATRA inhibited the mRNA expression of CDK6, CDK4, cyclin D3 and cyclin D1; NaB inhibited the mRNA expression of CDK2, cyclin D2 and cyclin D1; ATRA and NaB inhibited the mRNA expression of CDK6, CDK4, CDK2, cyclin D1, cyclin D2 and cyclin D3; ATRA and/or NaB both stimulated p21 expression at the mRNA levels. It is concluded that the NaB effect on cell proliferation/differentiation may be linked to its ability to induce expression of p21 mRNA and inhibit the cyclin D-CDK complexes. These observations support the claim that NaB has the synergistic effect with ATRA.