Acute adrenal crisis after orthopedic surgery for pathologic fracture

BACKGROUND: Adrenal crisis after surgical procedure is a rare but potentially catastrophic life-threatening event. Its manifestations, such as hypotension, tachycardia, hypoxia, and fever mimic the other more common postoperative complications. Clinical outcome is dependent upon early recognition of the condition and proper management with exogenous steroid administration. CASE PRESENTATION: We report a 75-year-old man who presented with shock immediately after surgery for a femoral fracture from lung cancer metastasis. Anemia and severe hyponatremia were detected. Despite adequate fluid resuscitation, nonspecific symptoms including hypotension, tachycardia, hypoxia, fever and confusion occurred. Emergent CT revealed enlarged bilateral adrenal glands. Under the diagnosis of adrenal crisis due to metastatic infiltration of adrenal glands, the patient was treated with appropriate steroid replacement resulting in rapid improvement and recovery. CONCLUSION: We describe a case of adrenal crisis caused by the lack of adrenal reserve based on metastatic involvement and surgical stress, the first published case of adrenal crisis after surgery for a pathologic fracture from lung cancer metastasis. Surgeons treating pathologic fractures should be aware of this complication and familiar with its appropriate therapy because of increasing opportunity to care patients with metastatic bone tumors due to recent advances in cancer treatment

Long-term efficacy and safety of tucidinostat in patients with relapsed or refractory peripheral T-cell lymphoma: final analysis of phase IIb results

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Oral HDAC inhibitor tucidinostat in patients with relapsed or refractory peripheral T-cell lymphoma: phase IIb results

Tucidinostat (formerly known as chidamide) is an orally available, novel benzamide class of histone deacetylase (HDAC) inhibitor that selectively blocks class I and class IIb HDAC. This multicenter phase IIb study aimed to investigate the efficacy and safety of tucidinostat, 40 mg twice per week (BIW), in patients with relapsed/refractory (R/R) peripheral T-cell lymphoma (PTCL). The primary endpoint was overall response rate (ORR) assessed by an independent overall efficacy review committee. Between March 2017 and March 2019, 55 patients were treated, and 46 and 55 were evaluated for efficacy and safety, respectively. Twenty-one of 46 patients achieved objective responses with an ORR of 46% (95% confidence interval : 30.9-61.0), including five patients with complete response (CR). Responses were observed across various PTCL subtypes. In angioimmunoblastic T-cell lymphoma, there were two CR and five partial responses (PR) among eight patients, achieving an ORR of 88%. The disease control rate (CR + PR + stable disease) was 72% (33/46). The median progression-free survival, duration of response, and overall survival were 5.6 months, 11.5 months, 22.8 months, respectively. The most common adverse events (AE) (all grades) were thrombocytopenia, neutropenia, leukopenia, anemia, and diarrhea. The grade ≥3 AE emerging in ≥20% of patients included thrombocytopenia (51%), neutropenia (36%), lymphopenia (22%), and leukopenia (20%). Importantly, most of the AE were manageable by supportive care and dose modification. In conclusion, the favorable efficacy and safety profiles indicate that tucidinostat could be a new therapeutic option in patients with R/R PTCL (clinicaltrials gov. Identifier: NCT02953652)

Acute Myeloid Leukemia Presenting as Subcutaneous and Epidural Granulocytic Sarcoma Inside and Outside of the Frontal Bone

An 18 year-old male was admitted to our hospital suffering from a large tumor which was located at the right frontal bone. He was diagnosed to have acute myeloid leukemia (AML) with granulocytic sarcoma (GS). A chromosomal analysis showed t(8; 21), and a flow cytometric analysis demonstrated the leukemic cells to be positive for CD56. Systemic chemotherapy and radiation therapy to the GS, but the patient experienced a relapse in the lumbar vertebrae. He underwent an umbilical-cord blood stem cell transplantation, however, he died 7 months thereafter. GS is a localized tumor consisting of leukemic myelolasts, which is generally observed as a complication of either AML, myelodysplastic syndrome, or myelobproliferative disorders. We herein report this case due to its rarity, even though various sites of GS have been reported

Polycythemia Vera Terminating in Refractory Ascites

A 64-year-old woman,with more than a 20 year history of polycythemia vera(PV),developed portal hypertension,myelofibrosis and extramedullary hematopoiesis accompanied by massive ascites. Portal hypertension resulted not only from infiltration of the liver sinusoids by hematopoietic cells but also from nodular regenerative hyperplasia of the liver. Wright-stained smears of ascites samples consisted of mesothelial cells and macrophages. However,cultures of mononuclear cells from the ascites showed the presence of hematopoietic progenitor cells including megakaryocyte colony formation and burst forming units. The JAK2-V617F mutation was positive in granulocytes. Contrary to other reports, radiation therapy was not effective and severe myelosuppression continued for more than one month. We present the unusual clinical course for this case of PV and discuss the pathophysiology of refractory ascites

Der(2)t(2;11)(p21;q23), a Variant form of t(2;11), in Biphenotypic Acute Leukemia with T Lymphoid Lineage and M yeloid Lineage Differentiation

We describe a patient with biphenotypic acute leukemia (BAL) with T-lymphoid lineage and myeloid lineage differentiation［BAL (T/M)］. Cytogenetic analysis revealed complex chromosomal abnormalities, including der(2)t(2;11)(p21;q23). Neither leukemia cells nor T-cell receptor gene rearrangements were detected in the bone marrow samples after four courses of high dose cytosine arabinoside regimen. However, der(2)t(2;11)(p21;q23) anomaly persisted in most of metaphases. Fluorescence in situ hybridization (FISH)analysis with a probe for MLL did not detect the split signal. Forty-five cases of hematological disorder with t(2;11)(p21;q23) abnormality have been previously reported. The majority of such cases have been classified as myelodysplastic syndrome(MDS) or acute myeloid leukemia (AML). This is the first case BAL (T/M) associated with a t(2;11)(p21;q23) anomaly