Acute Promyelocytic Leukemia Presenting Research Articles

Acute promyelocytic leukemia presenting with basophilia: diagnostic features and clinical observation

Acute promyelocytic leukemia (APL) is a blood tumor characterized by aggressive clinical progression with characteristic clinical and laboratory signs (typical tumor cell morphology, severe hemorrhagic syndrome, hemorrhages with hematoma formation, excessive fibrinolysis, disseminated intravascular coagulation syndrome) but modern approaches to therapy potentially make this disease fully curable. APL is considered one of the most studied subtypes of acute leukemia and is characterized by abnormal proliferation of promyelocytes in the bone marrow. The morphologic picture of APL is quite diverse, but an increased basophil count represents a rare manifestation that requires further study and understanding of its significance. Currently, there are limited scientific data on the clinical course and response to standard therapy in patients with APL presenting with basophilia.

AML-503 An Unusual Presentation of Acute Promyelocytic Leukemia (APML): Complex Cytogenetic Isochromosome 17 and WT1 Gene Mutation in the Absence of PML-RARA Fusion Protein

AML-503 An Unusual Presentation of Acute Promyelocytic Leukemia (APML): Complex Cytogenetic Isochromosome 17 and WT1 Gene Mutation in the Absence of PML-RARA Fusion Protein

A Case of Acute Promyelocytic Leukemia Presenting as Sternal Infiltrate

Granulocytic sarcoma (GS) or myeloid sarcoma (MS) is a localized extramedullary tumor composed of immature myeloid cells which can develop de novo or concurrently with acute myeloid leukemia (AML), myeloproliferative disorders (MPN) or myelodysplastic syndrome (MDS). It can present before, along with or after the diagnosis of acute myeloid leukemia, MPN, MDS or may present as a manifestation of relapse in a previously treated AML patient. The occurrence of MS in an acute promyelocytic leukemia (APL) patient is a very uncommon clinical event especially as initial or primary presentation. APL is often associated with a life threatening coagulopathy making prompt diagnosis and initiation of therapy critical. Unusual clinical localization may lead to misdiagnosis, or delayed diagnosis and treatment. Hence being aware of such atypical presentations of APL is crucial. Here we report a patient with acute promyelocytic leukemia who presented with sternal infiltrate but without any systemic symptoms. The diagnostic challenges are of particular interest given the unusual clinical presentation along with atypical morphology of the abnormal promyelocytes.

203 - Acute promyelocytic leukemia presenting with acute stroke

203 - Acute promyelocytic leukemia presenting with acute stroke

Acute Promyelocytic Leukemia Masquerading as Sero-negative Polyarthritis: Case Report

AbstractMusculoskeletal manifestations as the sole presentation in acute leukemia is rare in adults. Acute promyelocytic leukemia (APML) is a subtype of acute myeloid leukemia (AML) with reported incidence of 10 to 15% of total AML cases. APML presenting as polyarticular arthritis has never been reported in the literature. We present an interesting case of 20-year-old male patient who manifested with polyarticular arthritis mainly of small joints as the initial presentation, followed by pancytopenia and eventually was diagnosed as a case of APML on bone marrow morphology and molecular analysis for PML-RARα transcript. He was successfully treated with all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO). Arthritis also resolved with complete remission of APML. Arthritis in a case with pancytopenia should promptly be evaluated prior to treatment with steroids and anti-metabolites. Arthritis can be a presenting manifestation of APML and responds to prompt management of leukemia as in other cases of leukemic arthritis.

Variant Acute Promyelocytic Leukemia Presenting Without Auer Rods Highlights the Need for Correlation with Cytogenetic Data in Leukemia Diagnosis.

Variant acute promyelocytic leukemia (vAPL) is a rare leukemia characterized by rearrangement between RARα and a non-PML partner gene. This type of leukemia can be difficult to recognize by histomorphologic evaluation, particularly in patients with few or no Auer rods, and by flow cytometry, but it can be identified by distinct cytogenetic features. Herein, we report on a patient with vAPL with t(11;17)(q23;q21) who presented an initial diagnostic challenge. Detailed flow cytometry findings are presented for this rare entity. Our case study also presents novel treatment (chemotherapy in combination with venetoclax) chosen based on mechanistic data from preclinical studies.

Abdominal emergency - Acute promyelocytic leukaemia presenting as a corpus luteal cyst

Acute promyelocytic leukaemia (APML) is known to present with disseminated intravascular coagulopathy (DIC) but as a Corpus luteal cyst hemorrhage however, it is rarely seen at presentation. This case is the first to describe a patient with APML who presented with ruptured corpus luteum without ovarian tissue infiltration with blasts, leading to hemoperitoneum posing as life threatening acute abdominal emergency in a woman belonging to child bearing age. The case is a singular case where APL has presented in such a rare manner.

Ethnic differences in acute promyelocytic leukaemia between New Zealand Polynesian and European patients

ABSTRACT Ethnic differences in haematologic malignancies remain poorly elucidated, hence research in this area is important. This was a retrospective study into potential ethnic disparity in the presentation and outcomes of acute promyelocytic leukaemia (APL) between New Zealand (NZ) Polynesian and European patients. Data were analysed for patients treated at Auckland City Hospital (ACH; n = 55) and recorded in the New Zealand Cancer Registry (NZCR; n = 173), both for the period 2000–2017. We found that Polynesian patients treated at ACH presented at a younger age than European (P = 0.005), showed higher blast counts (P = 0.033), and a marginally higher prothrombin ratio (P = 0.02). Treatment with all-trans retinoic acid (ATRA) was started faster in Polynesian patients than European (P = 0.021), suggesting Polynesians were sicker at presentation but were managed accordingly. There were no differences in bleeding events, transfusion requirements and early deaths during the first month of treatment. Long-term survival was also similar. Data extracted from the NZCR confirmed NZ Polynesian patients with APL were younger than European (P < 0.001), but long-term survival was similar (P = 0.920). In summary, this study indicates a discrepancy in the presentation and severity of APL between NZ Polynesian and European patients but treatment initiation was rapid with no difference in outcomes. The distinctive features of APL in NZ Polynesians raise the possibility of a predisposing genetic factor or a different risk factor profile, elucidation of which is important for all patients with APL.

Donor-derived acute promyelocytic leukemia presenting as myeloid sarcoma in a transplanted kidney

Donor-derived acute promyelocytic leukemia presenting as myeloid sarcoma in a transplanted kidney

Deluging Thrombosis: An Unusual Presentation of Acute Promyelocytic Leukemia

AbstractAcute promyelocytic leukemia (APML) patients are prone to thrombosis. However, thrombosis at presentation is rare in APML. Our patient presented with thrombosis and cytopenia, and the clinical diagnosis was of paroxysmal nocturnal hemoglobinuria. However, subsequent peripheral blood smear and bone marrow study confirmed the diagnosis of APML. The patient was successfully managed with anticoagulation, arsenic trioxide, and all-trans retinoic acid.

Acute promyelocytic leukemia presenting with atypical basophils.

We describe a case of acute promyelocytic leukemia with circulating aberrant basophils. Recent studies have shown that basophilic differentiation is not uncommon in APL and likely under‐recognized in morphologic and immunophenotypic assessments.

Cerebral Thrombosis As an Initial Presentation of Pediatric Acute Promyelocytic Leukemia

Disseminated intravascular coagulopathy and bleeding are common manifestations of acute promyelocytic leukemia(APL) which are both associated with significant mortality and morbidity. However APL related thrombosis is very rare and majority of the reported cases are adults in the literature. In the past 14 years, 14 cases with APL were found in our hospital database.There were 6 female and 8 male children with a mean age of 10.7±4.6 years (2-17y). Here, we report two pediatric APL case who presented with cerebral thrombosis at the initial presentation. 8/14(57%) had disseminated intravascular coagulation at the initial presentation and one patient had intracardiac and cerebral sinovenous thrombosis during the induction period. Case 1:A 13-year-old boy was admitted to our hospital with a complaint of headache for two days. His physical examination revealed a papilledema and cranial MRI did show sinovenous thrombosis. At the time of the evaluation his WBC count was found to be low and there were myeloid blasts on his peripheral smear,bone marrow aspiration was done and diagnosed as translocation t(15;17)positive APL. He remain in remission more than 4 years without any event however deceased after relapse. Case 2: A 8-year-old boy was admitted to our hospital with a complaint of right hemiparesis, there was a thrombotic occlusion of the left middle cerebral artery associated cerebral infarct on his MRI.His WBC was 5.9x109/l with 76% blasts and bone marrow aspiration and flow-cytometry were both compatible with APL. Patient died after 2 weeks of admission because of thrombosis and bleeding. The incidence of APL related thrombosis was 4.5 and 5.6% in two large adult cohorts. Both arterial and venous thrombosis may ocur in patients with APL. To the best of our knowledge there were limited number of pediatric APL case reports in the literature who presented with thrombosis. In our small group of pediatric APL patients 14% presented with thrombosis and the total number of thrombotic events were found to be 28% during the follow up. Disclosures No relevant conflicts of interest to declare.

Disseminated Intravascular Coagulation With Purpura Fulminans Presentation of Acute Promyelocytic Leukemia.

A 47-year-old male presented to the emergency department with 12 hours of nausea, vomiting, abdominal pain, and a widespread skin eruption with mottled, irregular, purpuric lesions with subsequent rapid decompensation. Laboratory analysis revealed thrombocytopenia, bandemia, elevated metamyelocytes, abnormal coagulation panel, decreased fibrinogen, elevated fibrin split products, renal dysfunction, bacterial rods, dohle bodies, and toxic granulation. Acute promyelocytic leukemia (APML) was confirmed via bone marrow biopsy, flow cytometry, and fluorescence in situ hybridization analysis. Disseminated intravascular coagulation (DIC) may be the initial presentation of APML. When treated promptly, APML can achieve high remission rates; however, conditions such as DIC continue to increase mortality requiring early recognition to improve survival rates.

An unusual case of acute promyelocytic leukemia manifesting with pulmonary embolism

Venous thromboembolism is four fold greater in patients with cancer. 5-20% of acute promyelocytic leukemia is M3 (Hypergranular promyelocytic leukemia) variety. Hemorrhagic complications are more common than thrombosis; Coagulopathy in acute promyelocytic leukemia is a very complex disorder and is under reported. Here is a case report of a rare presentation of acute promyelocytic leukemia manifesting with pulmonary embolism.

Acute Promyelocytic Leukemia Presenting Unusual Case with Additional Cytogenetic Abnormality

Acute Promyelocytic Leukemia Presenting Unusual Case with Additional Cytogenetic Abnormality

Myelodysplasia-related acute myeloid leukemia and acute promyelocytic leukemia: concomitant occurrence of two molecularly distinct diseases.

Concurrent presentation of acute promyelocytic leukemia (APL) with other hematologic diseases in the absence of previous chemotherapy or ionizing radiotherapy treatment is very rare. We present a case of simultaneous occurrence of APL with myelodysplastic syndrome (MDS)-related acute myeloid leukemia (AML). A 43-yearold female presented with 3 month of history fatigue, night sweats, chills and pancytopenia. Bone marrow aspirate and biopsy demonstrated 20% myeloid blasts with dysplastic changes admixed with abnormal promyelocytes. Cytogenetic analysis showed tetraploidy and deletion in chromosomes 5q and 7q and polymerase chain reaction showed presence of PML/RARA mRNA transcripts, confirming the presence of concurrent APL and MDS-related AML. Induction chemotherapy with cytarabine and daunorubicin was initiated along with all-trans retinoic acid. This is the first case to be reported in the literature of concurrent occurrence of APL with MDS-related AML. Treatment with 7 + 3 regimen and ATRA was successful in inducing complete remission.

Status epilepticus as initial presentation of acute promyelocytic leukemia

Coagulopathy is a complex finding in acute promyelocytic leukemia (APL), and it may occur due to interplay of the various factors. Neurological manifestations are rare as the initial presentation in APL. This case illustrates status epilepticus as the initial presentation in this disease. Atypical cells on peripheral smear led to the diagnosis of leukemia. This report demonstrates that the patient of APL can present as status epilepticus.

Acute Promyelocytic Leukemia Presenting as Focal Neurologic Findings and Deteriorating Mental Status

BackgroundAcute promyelocytic leukemia (APL) is a rare but particularly malignant form of acute leukemia that is characterized by a rapid progression to fatal hemorrhage. Survival rates of patients with APL have increased with the introduction of all-trans retinoic acid (ATRA), but early deaths caused by hemorrhage still persist. Case ReportA man with undiagnosed APL presenting with focal neurologic findings and deteriorating altered mental status caused by an intracranial hemorrhage is discussed. Why Should An Emergency Physician Be Aware of This?It is important to consider APL when diagnosing etiologies for intracranial hemorrhage. In addition to standard care, early administration of ATRA is recommended upon clinical suspicion of the disease.

Acute Promyelocytic Leukemia Presenting with Extensive Fibrosis: Report of an Uncommon Variant

Acute Promyelocytic Leukemia Presenting with Extensive Fibrosis: Report of an Uncommon Variant

Acute Promyelocytic Leukaemia Presenting as Myeloid Sarcoma

Acute Promyelocytic Leukaemia Presenting as Myeloid Sarcoma