Acute Myeloid Leukemia Presenting Research Articles

Successful Treatment of Relapsed Pediatric Acute Myeloid Leukemia Presenting as Central Nervous System Myeloid Sarcoma: A Single-Institution Case Series.

Relapsed acute myeloid leukemia presenting as an isolated central nervous system myeloid sarcoma (CNS MS) is very rare and generally entails poor outcomes. CNS MS treatment is not well defined and can include systemic chemotherapy, intrathecal chemotherapy, radiation therapy, or hematopoietic stem cell transplant. Thiotepa, vinorelbine, topotecan, and clofarabine (TVTC) has been successful for reinduction therapy in relapsed/refractory leukemia to induce remission before hematopoietic stem cell transplant. There is no published evidence of TVTC being utilized for CNS MS. In this series, we report 2 symptomatic patients with isolated CNS MS at relapse who demonstrated near complete resolution after reinduction with TVTC and additional intrathecal chemotherapy.

Impact of Leukapheresis and Time to Chemotherapy on Outcomes of Newly Diagnosed Patients (pts) with Acute Myeloid Leukemia (AML) Presenting with Hyperleukocytosis: An Analysis from a Large International Patient Cohort

Impact of Leukapheresis and Time to Chemotherapy on Outcomes of Newly Diagnosed Patients (pts) with Acute Myeloid Leukemia (AML) Presenting with Hyperleukocytosis: An Analysis from a Large International Patient Cohort

Rare Initial Presentation of Acute Myeloid Leukemia as Facial Palsy

Rare Initial Presentation of Acute Myeloid Leukemia as Facial Palsy

Intra‐oral noma‐like lesion associated with acute myeloid leukaemia: a case report

AbstractAcute myeloid leukaemia is a malignancy of the white blood cells that can develop in both children and adults with rapid development and possible intra‐oral manifestations.This report describes a case involving a 50‐year‐old patient who was referred to the Emergency Department of a University Dental Hospital complaining of severe intra‐oral ulceration, spontaneous intra‐oral bleeding and generalised fatigue and malaise. Examination revealed the existence of an erythematous intra‐oral necrotising, noma‐like, ulceration in the posterior area of the right buccal mucosa. The patient was diagnosed with acute myeloid leukaemia.Acute myeloid leukaemia develops rapidly and the initial symptoms may be non‐specific. Patients may demonstrate intra‐oral manifestations; most common symptoms include spontaneous gingival bleeding, gingival enlargement and acute ulceration. Ulceration with necrotising (noma‐like) characteristics is a rare oral manifestation of acute myeloid leukaemia, the cause of which is yet unknown. Fewer than 10 cases have been reported in the international literature.Intra‐oral pathology can be an early indicator of significant medical pathology. A dental practitioner is likely to be the first point of contact for patients with acute myeloid leukaemia due to the unusual or painful nature of the intra‐oral pathology; identification of suspicious signs and symptoms can lead to early detection and treatment.Clinical relevance Leukaemia may present initially to dental practitioners because of a high incidence of early intra‐oral signs and symptoms. A high index of suspicion for underlying systemic disease will lead to prompt onward referral for specialist investigations.Oral presentations may include spontaneous gingival bleeding and gingival enlargement. A rare oral presentation of acute myeloid leukaemia is deep and severe oral ulceration presenting as a noma‐like lesion, which we present here.

Ophthalmic Manifestations In Patients of Acute Leukaemia Presenting to A Tertiary Care Centre In Western Rajasthan

Purpose:- To determine the prevalence and pattern of ophthalmic manifestations in patients of Acute Myeloid Leukemia (AML) and Acute Lymphoid Leukemia (ALL) presenting to a tertiary care centre in Western Rajasthan.

Hispanic ethnicity is associated with younger age at presentation but worse survival in acute myeloid leukemia.

SEER data and a Bronx validation cohort demonstrate that Hispanics present with AML at younger age but have shorter survival than whites.Increased frequency of high-risk mutations in Hispanics provides a potential biologic explanation for poorer outcomes in Hispanics.

RUNX1-RUNX1T1-positive acute myeloid leukaemia presenting as bilateral proptosis and multiple cranial nerve palsy

We describe a unique presentation of acute myeloid leukaemia (AML) with myeloid sarcoma (MS), manifested as proptosis with multiple cranial nerve palsies in a 9-year-old boy. MRI of the brain...

Proptosis in orbital myeloid sarcoma: A precursor to acute leukaemia

Myeloid sarcoma is a rare, aggressive presentation of acute myeloid leukemia which tends to affect bones and soft tissue. It is usually seen in children, and has a poor prognosis. We report a case of orbital myeloid sarcoma presenting with unilateral proptosis in a young adult who subsequently developed acute myeloid leukaemia. An 18-year-old male presented with sudden, progressively increasing right eye swelling over two weeks. Examination revealed right eye proptosis, with a positive relative afferent papillary defect and a right eye visual acuity of 6/60, pinhole 6/18. The right fundus showed optic disc hyperemia, tortuous veins and choroidal folds. The left eye was normal. On initial examination, computed tomography (CT) of the brain and orbit showed an enhancing mass at the lateral aspect of the right orbit measuring 3.8 × 2.1 cm, with optic nerve displacement. Right lateral orbitotomy and excisional biopsy yielded a tissue diagnosis of myeloid sarcoma. The peripheral blood smear was normal at this time. The patient defaulted treatment but presented again three months later with right eye massive proptosis and loss of vision. A repeated CT scan showed a large lobulated mass measuring 104.5mm × 87.21mmm × 77.25mm occupying the right orbit, with destruction of the bony orbital walls and intracranial extension. A repeat full blood picture and bone marrow aspiration showed 30% blast cells, consistent with the diagnosis of acute myeloid leukemia. Patient was started on chemotherapy and radiotherapy. The tumour reduced in size after five cycles, but unfortunately, patient passed away due to brain metastases.

Bilateral Proptosis: An Atypical Presentation of Acute Myeloid Leukaemia

Bilateral Proptosis: An Atypical Presentation of Acute Myeloid Leukaemia

Paraplegia as initial presentation of acute myeloid leukemia: A case report

Paraplegia as initial presentation of acute myeloid leukemia: A case report

Ocular Granulocytic Sarcoma as an Initial Clinical Presentation of Acute Myeloid Leukemia Identified on Flurodeoxyglucose Positron Emission Tomography/Computed Tomography.

Granulocytic sarcoma (GS) or chloroma, rare extramedullary manifestation of acute myeloid leukemia and not infrequently, can be presenting clinical feature. Multiple studies have demonstrated the clinical utility of fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in early detection and follow-up assessment of GS after chemotherapy. Commonly involved areas include bones, lymph nodes, breasts, and skin and not uncommonly, the disease can be multifocal. We present a rare case of ocular GS, where FDG-PET/CT in addition to the identifying the ocular mass, revealed multiple clinically occult extramedullary lesions.

Prognosis of Patients with Standard- or Intermediate-Risk Acute Myeloid Leukemia Presenting with Residual Disease after One Induction Cycle Is Improved If Remission Is Achieved Following Re-Induction Given By Day 14 Compared to Second-Line Therapy Given 24-40 Days after Induction

Introduction: Failure to achieve remission following standard induction is a poor prognostic sign in patients with acute myeloid leukemia (AML). However, as there is no consensus on the optimal number of induction cycles and/or the best indication and timing for re-induction, the definition of refractory AML is still debatable. A clinically relevant definition of refractoriness should be associated with poor long-term prognosis. This is of particular importance for patients who eventually achieve remission (CR1) and in whom referral for allogeneic stem cell transplantation (alloSCT) in CR1 may be controversial. It was previously reported that AML patients who achieved CR1 following re-induction given on day 14 of therapy, had a long-term survival identical to that of patients who achieved CR1 after one induction cycle. To the best of our knowledge, no data are available regarding the prognosis of patients who achieved CR1 after a second cycle of intensive chemotherapy, administered at the end of neutropenic period.The current study compared the prognosis of AML patients who achieved CR1 after one induction cycle to that of patients who achieved CR1 following a second cycle of chemotherapy given either as re-induction by day 14 or as a second-line therapy administered on days 24-40 of the first induction.Patients and Methods: Data of all leukemia patients admitted to the Rambam Medical Center between the years 1993 and 2015 were retrospectively collected. Adults diagnosed with de-novo AML who achieved CR1 following one or two cycles of therapy were included in the study. Patients diagnosed with APL or high-risk AML, as defined based on a poor-risk karyotype or presence of FLT3-ITD mutation, were excluded from the analysis. It is common practice at our center to assign patients for re-induction according to day 14 bone marrow (BM) results. Yet, for various reasons, this paradigm is not followed in some patients. Overall survival (OS), leukemia-free-survival (LFS), and death rates were compared in the study participants. Multivariate analysis for prediction of death, including the use and timing of second-line therapy, was performed.Results: Six hundred and forty three de novo AML patients were treated at Rambam during the study period. After exclusion of patients with high-risk AML, 492 individuals were left. Of them, 242 patients achieved CR1 after one cycle of induction (control group), 53 after induction + re-induction administered by day 14 (re-induction group) and 38 achieved CR1 after induction followed by second-line therapy prescribed on days 24-40 (second-line group).Median OS was 25.9, 23.5 and 15.1 months for patients in control, re-induction and second-line groups, respectively. Median LFS was 14.7, 10 and 8.16 months for the corresponding groups, respectively. Differences in OS and LFS were statistically significant between the control and second-line groups (p<0.0001 for both comparisons), and between the re-induction and second-line groups (p=0.007 for OS; p=0.001 for LFS). Differences in OS and LFS between the control and re-induction groups were insignificant (p=0.46, p=0.49). In multivariate analysis including all patients, age and cytogenetics were the only significant factors to predict survival. When the 40 patients presenting with standard-risk (favorable) cytogenetics were excluded, age and treatment were the only significant factors to predict survival in multivariate analysis.Conclusions: Despite previous reports showing similar outcomes in patients who achieved CR1 post re-induction and those achieving CR1 with one induction cycle, some physicians are reluctant to start re-induction as early as day 14. We herein report a significantly better prognosis in patients achieving CR1 after re-induction given by day 14 compared to those achieving CR1 after a second cycle of therapy prescribed on days 24-40. These data may have 2 practical implications. First, in AML patients with residual disease, an effort should be made to start re-induction as early as day 14. Second, for intermediate-risk patients, a failure to achieve CR1 after one induction cycle should not alter post-remission decisions, if CR1 was achieved with re-induction by day 14. This may not be true for patients who achieved CR1 with second-line therapy prescribed on days 24-40. Our study was not powered to examine whether alloSCT could improve the outcome of these refractory AML patients. DisclosuresNo relevant conflicts of interest to declare.

Clinical Features and Outcomes of Isolated Myeloid Sarcoma in the United States: Analysis Using a National Dataset

IntroductionIsolated myeloid sarcoma is a rare form (<1%) of acute myeloid leukemia presenting as extramedullary tumor. Contemporary clinical data are mostly limited to institutional case series. Using the National Cancer Database, the largest public cancer registry covering >70% of all newly diagnosed cancers in the United States, we determined the patterns of anatomical presentation and clinical outcome of myeloid sarcoma.MethodsWe identified patients with a histologically confirmed diagnosis of isolated myeloid sarcoma from 2004-2013 using International Classification of Diseases for Oncology version 3 (ICD-O-3) code: 9930. To allow at least 1 year of follow-up, only patients diagnosed from 2004-2012 were included in the survival analysis using Kaplan-Meier estimates.ResultsA total of 746 patients were included in the study. The median age of patients was 59 years (range, 41 to 73) and 56% were males. The anatomical distribution and median overall survival of patients are depicted in the Table. The top 3 most common sites of presentation were connective/soft tissues (31.3%), skin/breast (12.3%), and digestive system (10.3%). Compared to other races, Blacks were more likely to have presentation in bones/joints (11.8% vs 6.3% in others), lymph nodes/spleen (22.1% vs. 9%), and less likely in skin/breast (4.4% vs. 13.8%). Asians were more likely to present with cardiopulmonary/mediastinal disease as compared to other races (13.6% vs. 4.2%). According to outcomes, we can categorize the patients into 3 groups: good (median overall survival >30 months: reproductive and digestive systems), intermediate (median overall survival 15-30 months: head/neck and kidney/bladder/retroperitoneum/adrenal), and poor (median overall survival <15 months: nervous system, connective/soft tissue, and bones/joints). There was no significant difference in overall survival between males and females (P =0.06). Among the races, Blacks had the worst overall survival (P =0.02; Figure).ConclusionsThis is the largest registry-based study on isolated myeloid sarcoma in the United States. Isolated myeloid sarcoma has a diverse anatomic clinical presentation and the overall survival varied significantly according to sites of presentation and racial subgroups. The results of our study may aid the prognostication of patients for treatment decision making and in the understanding of the biological differences by anatomic sites of presentation. [Display omitted] [Display omitted] DisclosuresAl-Kali:Onconova Therapeutics, Inc.: Research Funding; Celgene: Research Funding.

Leukemia Blast Crisis: A Simulation Case for Residents.

Oncologic emergencies are life-threatening and often require advanced team-management skills as well as a mastery of disease processes and therapeutic interventions. Simulation of an oncologic emergency case is a useful way to experience, reflect on, and practice these skills. This case involving a simulated patient in blast crisis was created as part of our Emergency Medicine (EM) Resident Simulation Curriculum at the Perelman School of Medicine at the University of Pennsylvania. This case is based on an actual patient seen in our emergency department and highlights specific teaching points and potential pitfalls in treatment algorithms. It details a 40-year-old female with history of acute myeloid leukemia presenting with fatigue for 2 days, tachycardia, and labored breathing. The patient develops worsening respiratory distress if not intervened upon and progresses to pulseless electrical activity arrest. Lab work is notable for markedly elevated white blood cell count and acidosis. A SimMan or SimMan3G is required for the simulation. The associated debriefing materials are Included in this educational resource. Evaluation of learner performance is mapped to the EM Milestones. Eighteen of the 20 (90%) EM residents that participated in this simulation, responded to the Likert-scale postsession survey. Of those who responded, 83% agreed or strongly agreed that the case was realistic and 89% agreed or strongly agreed that the case was useful. The simulation venue offers a unique opportunity to address team dynamics as well as provide a forum for didactic learning as it is often difficult to debrief a critical case while working in real-time patient care settings.

Severe Lactic Acidosis as a Fatal Presentation of Acute Myeloid Leukemia

SESSION TITLE: Fellow Case Report Poster - Critical Care I SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Often patients with acute myeloid leukemia (AML) present with fatigue, infections or bleeding due to cytopenias. Lactic acidosis as the presenting manifestation of acute myeloid leukemia is rare. CASE PRESENTATION: 75 year old female with a history of right stage III breast cancer (2013) with total mastectomy s/p chemotherapy and radiation therapy, presented to the emergency room with a one week history of flu like symptoms with increasing shortness of breath and altered mental status for 1 day. Vital signs showed a pulse rate100 bpm, BP 120/76 mmHg, respiratory rate 26 rpm with a saturation of 97% on room air. On exam she was ill appearing with diffuse abdominal tenderness and agitation. Lung and cardiac exam were unremarkable. Laboratory workup revealed a white blood cell count 194,000/uL, absolute monocyte count of 79,000/ uL (>20% blasts), hemoglobin 9.8 g/dL, platelet count 118 k/uL, serum creatinine was 1.98 mg/dL, liver enzymes were elevated (AST 1,486 U/L, ALT 2,404U/L, alkaline phosphatase 245 U/L). ABG revealed pH 6.73, pCO2 26 mmHg, pO2 91 mmHg, bicarbonate 8 mmol/L, anion gap 39 mmol/L and lactate 19.8 mmol/L. Chest X-Ray, CT brain and CT abdomen/pelvis showed no acute pathology. Blood cultures, urine cultures and urine toxicology screen were negative. She received IV fluid resuscitation, empiric broad-spectrum antibiotics and vasopressor support. The patient required intubation for worsening respiratory failure and mental status. Hematology oncology evaluated the patient due to the concern for acute leukemia with leukostasis. Peripheral smear showed acute numerous promonocytes consistent with an acute monocytic leukemia. Vasopressor requirements continued to increase and the patient expired within 24 hrs of presentation to the Emergency Department. DISCUSSION: The liver dysfunction alone in the absence of hypotension, hypoxia or infection would not explain the degree of lactic acidosis in our patient. Lactic acidosis is a rare but potentially fatal complication of AML. The pathogenesis of lactic acidosis in leukemia is unclear but is likely due to the anaerobic glycolysis by leukemic cells; tissue hypoxemia from leukostasis or thiamine and riboflavin deficiency. CONCLUSIONS: It is important to be cognizant that acute leukemia can present with lactic acidosis. Lactic acidosis in the presence of hematologic malignancy carries a poor prognosis. Reference #1: Udayakumar N, Rajendiran C, Muthuselvan R. A typical presentation of acute myeloid leukemia. J Cancer Res Ther. 2006;2(2):82-4. DISCLOSURE: The following authors have nothing to disclose: Shruti Gadre, Pulkit Chaudhury, Hariom Joshi, Adriano Tonelli No Product/Research Disclosure Information

Acute Myeloid Leukemia Presenting as Galactorrhea

Acute myeloid leukemia (AML) presents with symptoms related to pancytopenia (weakness, infections, bleeding diathesis) and organ infiltration with leukemic cells. Galactorrhea is an uncommon manifestation of AML. We report a case of AML presenting with galactorrhea.

Transient spontaneous remission in congenital MLL-AF10 rearranged acute myeloid leukemia presenting with cardiorespiratory failure and meconium ileus

BackgroundNeonatal leukemia is a rare disease with an estimated prevalence of about one to five in a million neonates. The majority being acute myeloid leukemia (AML), neonatal leukemia can present with a variety of symptoms including hyperleucocytosis, cytopenia, hepatosplenomegaly, and skin infiltrates. Chromosomal rearrangements including mixed lineage leukemia (MLL) translocations are common in neonatal AML.Case presentationA female neonate born at 34 weeks gestation presented with cardiorespiratory failure, hepatosplenomegaly, pancytopenia, and coagulopathy. She required intensive care treatment including mechanical ventilation, high-dose catecholamine therapy, and multiple transfusions. Small intestinal biopsy obtained during laparotomy for meconium ileus revealed an infiltrate by an undifferentiated monoblastic, MLL-rearranged leukemia. No other manifestations of leukemia could be detected. After spontaneous clinical remission, lasting 5 months without any specific treatment, the patient presented with leukemia cutis and full-blown monoblastic leukemia. MLL-AF10-rearranged AML could be re-diagnosed and successfully treated with chemotherapy and hematopoietic stem cell transplantation.ConclusionsOur patient exhibited a unique manifestation of neonatal MLL-AF10 rearranged AML with cardiorespiratory failure and intestinal infiltration. It highlights the importance of leukemia in the differential diagnosis of neonatal distress, congenital hematological abnormalities, and skin lesions.

Acute Myeloid Leukemia Presenting with Sweet Syndrome: A Case Report and Review of the Literature

Acute Myeloid Leukemia Presenting with Sweet Syndrome: A Case Report and Review of the Literature

How I treat acute myeloid leukemia presenting with preexisting comorbidities

AbstractAcute myeloid leukemia (AML) is a devastating disease with an incidence that progressively increases with advancing age. Currently, only ∼40% of younger and 10% of older adults are long-term survivors. If untreated, the overall prognosis of AML remains dismal. Initiation of therapy at diagnosis is usually urgent. Barriers to successful therapy for AML are the attendant toxicities directly related to chemotherapy or those associated with inevitable aplasia. Organ dysfunction often further complicates such toxicities and may even be prohibitive. There are few guidelines to manage such patients and the fear of crossing the medico-legal abyss may dominate. Such clinical scenarios provide particular challenges and require experience for optimal management. Herein, we discuss select examples of common pretreatment comorbidities, including cardiomyopathy, ischemic heart disease; chronic renal failure, with and without dialysis; hepatitis and cirrhosis; chronic pulmonary insufficiency; and cerebral vascular disease. These comorbidities usually render patients ineligible for clinical trials and enormous uncertainty regarding management reigns, often to the point of withholding definitive therapy. The scenarios described herein emphasize that with appropriate subspecialty support, many AML patients with comorbidities can undergo therapy with curative intent and achieve successful long-term outcome.

Paraneoplastic pemphigus as a presentation of acute myeloid leukemia: Early diagnosis and remission

Skin lesions are frequently encountered in clinical practice which can be a presentation of systemic diseases not excluding an occult malignancy. Commonly reported paraneoplastic dermatologic manifestations include acanthosis nigricans, dermatomyositis, erythroderma, hypertrophic osteoarthropathy, Sweet syndrome, and paraneoplastic pemphigus (PNP).PNP is a rare autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphic skin eruptions, and associated underlying neoplasms most commonly non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, and Castleman disease. PNP is characterized on histopathology as dyskeratotic epithelial cells with acantholysis with a typical immunofluorescence staining pattern of direct and/or indirect staining of intercellular, basement membrane, and dermoepidermal junction with immunoglobulin-G and C3. PNP has been described to have poor prognosis with a mortality range of 75–90% and a mean survival of less than 1year.We describe a previously unreported case of PNP associated with acute myeloid leukemia (AML) where the patient presented with a nonhealing ulcer and hemorrhagic crusting on the face that did not respond to antimicrobials and steroids. Investigations revealed leukocytosis with peripherally circulating blasts. Skin biopsy revealed an evolving PNP and bone marrow biopsy confirmed evidence of AML. The patient underwent induction, consolidation, and then successful allogenic bone marrow transplantation with complete remission. The skin lesion, which was initially refractory to treatments, surprisingly resolved within 7days of starting induction chemotherapy.In this case, the skin lesion was a key factor in early diagnosis and instituting treatment for the underlying AML. Early intervention gave our patient a better outcome with an ongoing survival of 18months since diagnosis, maintaining complete remission.