The myeloid sarcoma treated by Venetoclax with hypomethylating agent followed by stem cell transplantation: rare case report

Abstract

Background Myeloid sarcoma (MS) is a very rare condition, develops both in patients with other hematological neoplasms, and as isolated tumor. MS of the gynecologic tract is extremely rare. An available literature data about diagnosis and management of MS is summarized in the article. The role of chemotherapy, radiation therapy, surgery and bone marrow transplantation in the treatment is discussed. Polychemotherapy and allogeneic bone marrow transplantation were suggested to be the optimal treatment strategy of MS of the gynecological tract. The use of new targeted agents results in promising clinical data. Case presentation We are presenting a rare clinical case of a MS of the uterine cervix with concomitant bone marrow involvement and describe all the peculiarities of the clinical course, diagnosis, and treatment. The patient received chemotherapy followed by allogeneic bone marrow transplantation. The pre-transplant therapy allowed us to perform allogeneic bone marrow transplantation with the deepest response possible: complete PET-negative and MRD-negative remission of the disease. Conclusions MS remains a subject of discussion regarding its diagnostic and therapeutic aspects. The use of novel targeting agents can be perspective option for patient with extramedullary disease.

Orbital myeloid sarcoma treated with low-dose venetoclax and a potent cytochrome P450 inhibitor

“…7 A standard dose of venetoclax (400 mg/day) and HMA was used successfully in a 49-year-old patient with newly diagnosed NPM1 systemic MS located in the cervix. 6 The patient received induction and consolidation chemotherapy with cytosine arabinoside and daunorubicin. The patient did not achieve complete remission because of an extramedullary lesion; therefore, a standard dose of venetoclax (400 mg/day) and azacytidine (75 mg/m 2 subcutaneously on days 1-7) was administered.…”

“…5 However, there are limited data on venetoclax in MS, and there are no data in the context of late post-haplo-HSCT relapse. 6 In addition, we took advantage of venetoclax interactions with CYP3A4 inhibitors and administered itraconazole to reduce the total dose of venetoclax by 75% (100 mg/day). 7 We present a case of orbital MS treated with low-dose venetoclax and a potent CYP3A4 inhibitor as a strategy to reduce the dose and cost Informed consent to publish the case report was obtained from the family.…”

The Progression of Chronic Myeloid Leukemia to Myeloid Sarcoma: A Systematic Review

“…To properly diagnose MS, symptomatic presentation is documented and followed up by tissue biopsy and radiological investigations [7]. Furthermore, treatment often involves chemotherapy approaches, bone marrow transplants, surgical excision, and targeted therapies tailored to the region affected by the tumor [7,34].…”

Long‐term remission of extramedullary cutaneous relapse of acute myeloid leukaemia (leukaemia cutis) treated with decitabine‐venetoclax

“…The infiltration of the skin by leukemic cells, called leukaemia cutis (LC), occurs in about 3% of patients with AML and is frequently associated with monocytic and myelomonocytic differentiation; though affecting only the skin, with localized or disseminated lesions, LC almost always represents a cutaneous manifestation of an underlying bone marrow disease and therefore systemic treatment should be administered [ 1 ]. A skin biopsy is necessary for establishing the correct diagnosis, and in patients presenting with a single skin lesion with symptoms of mass effect surgical intervention may be indicated before the systemic chemotherapy [ 3 ]. Until recently, regardless of bone marrow involvement, a Cytarabine–containing regimen has been the most reasonable approach to LC [ 4 ].…”

“…Targeted agents like BCL‐2 inhibitors (Venetoclax), hypomethylating agents (Decitabine and 5‐Azacitidine) and Gemtuzumab Ozogamicin have also been used successfully. In addition, eligible patients with or without bone marrow disease can receive hematopoietic stem‐cell transplantation (HSCT) [ 3 ]. Here we report a case of LC relapse, without bone marrow involvement, in a patient with AML who achieved and maintains a continuous complete remission (CR) with a long cyclic administration of Decitabine and Venetoclax (VEN‐DEC).…”