Acute Promyelocytic Leukemia Presenting Research Articles

Acute Budd-Chiari syndrome as an initial presentation of acute promyelocytic leukemia

Budd-Chiari syndrome (BCS) is the constellation of clinical signs and symptoms resulting from occlusion of two or more hepatic veins, often due to an underlying thrombophilic disorder. Acute myeloid leukemia has been rarely reported to be associated with hepatic vein thrombosis due to hyperleukocytosis, hyperfibrinolysis and disseminated intravascular coagulation. We report a case of acute promyelocytic leukemia where the clinical onset of the hematological disease was with acute BCS.

Rapidly progressing, fatal and acute promyelocytic leukaemia that initially manifested as a painful third molar: a case report

IntroductionAcute promyelocytic leukaemia, an uncommon and devastating subtype of leukaemia, is highly prevalent in Latin American populations. The disease may be detected by a dentist since oral signs are often the initial manifestation. However, despite several cases describing oral manifestations of acute promyelocytic leukaemia and genetic analysis, reports of acute promyelocytic leukaemia in Hispanic populations are scarce. The identification of third molar pain as an initial clinical manifestation is also uncommon. This is the first known case involving these particular features.Case presentationA 24-year-old Latin American man without relevant antecedents consulted a dentist for pain in his third molar. After two dental extractions, the patient experienced increased pain, poor healing, jaw enlargement and bleeding. A physical examination later revealed that the patient had pallor, jaw enlargement, ecchymoses and gingival haemorrhage. Laboratory findings showed pancytopaenia, delayed coagulation times, hypoalbuminaemia and elevated lactate dehydrogenase. Splenomegaly was detected on ultrasonography. Peripheral blood and bone marrow analyses revealed a hypercellular infiltrate of atypical promyelocytic cells. Cytogenetic analysis showing genetic translocation t(15;17) further confirmed acute promyelocytic leukaemia. Despite early chemotherapy, the patient died within one week due to intracranial bleeding secondary to disseminated intravascular coagulation.ConclusionThe description of this unusual presentation of acute promyelocytic leukaemia, the diagnostic difficulties and the fatal outcome are particularly directed toward dental surgery practitioners to emphasise the importance of clinical assessment and preoperative evaluation as a minimal clinically-oriented routine. This case may also be of particular interest to haematologists, since the patient's cytogenetic analysis, clinical course and therapeutic response are well documented.

A Case of Atypical Presentation of Acute Promyelocytic Leukemia

A Case of Atypical Presentation of Acute Promyelocytic Leukemia

Recurrent Acute Promyelocytic Leukemia Presenting As a Sacral Nerve Root Mass

Recurrent Acute Promyelocytic Leukemia Presenting As a Sacral Nerve Root Mass

Acute promyelocytic leukaemia presenting with a myeloid sarcoma of the tongue

Acute promyelocytic leukaemia presenting with a myeloid sarcoma of the tongue

Presentation of acute promyelocytic leukemia as granulocytic sarcoma

Granulocytic sarcoma (GS) is a localized tumor composed of immature myeloid cells. This extramedullary tumor can present before, concurrent with or after the diagnosis of acute myeloid leukemia. GS is extremely uncommon in acute promyelocytic leukemia (APL). As a proportion of patients never develop systemic disease, correct and timely diagnosis may be rather difficult, but is a prerequisite for optimal outcome. GS should be considered in the differential diagnosis of children with unusual bone lesions. We describe a patient with GS who presented with symptoms mimicking osteomyelytis or rheumatoid disease.

Relapse of acute promyelocytic leukemia presenting as granulocytic sarcoma in the hip

Relapse of acute promyelocytic leukemia presenting as granulocytic sarcoma in the hip

Morphologic heterogeneity of acute promyelocytic leukemia: Therapy-related acute promyelocytic leukemia presenting with FAB-M2 morphology

American Journal of HematologyVolume 81, Issue 6 p. 475-476 Letters and CorrespondenceFree Access Morphologic heterogeneity of acute promyelocytic leukemia: Therapy-related acute promyelocytic leukemia presenting with FAB-M2 morphology† S. Sinha, S. Sinha Department of Hematology/Oncology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MassachusettsSearch for more papers by this authorL. Aish, L. Aish Department of Pathology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MassachusettsSearch for more papers by this authorT.H. Oo, T.H. Oo Department of Hematology/Oncology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MassachusettsSearch for more papers by this author S. Sinha, S. Sinha Department of Hematology/Oncology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MassachusettsSearch for more papers by this authorL. Aish, L. Aish Department of Pathology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MassachusettsSearch for more papers by this authorT.H. Oo, T.H. Oo Department of Hematology/Oncology, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MassachusettsSearch for more papers by this author First published: 05 May 2006 https://doi.org/10.1002/ajh.20577Citations: 1 † Published as an abstract in the 2005 proceedings of the american society of clinical oncology (abstract 6741). AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume81, Issue6June 2006Pages 475-476 ReferencesRelatedInformation

Acute promyelocytic leukemia: an unusual cause of fatal secondary postpartum hemorrhage

Postpartum haemorrhage can rarely be associated with an underlying coagulation or haematological disorder. We wish to discuss a case of acute promyelocytic leukemia (APL) presenting as secondary postpartum hemorrhage (PPH), its clinical and pathological features and maternal outcome. We describe a 28-year-old woman who presented with secondary PPH accompanied by bleeding from gums, marked pallor, hematemesis, ecchymotic and purpuric spots all over the body, 8 days post-partum. Investigations revealed her to be having APL, a diagnosis not suspected by the referring clinic. She was given supportive therapy but died before chemotherapy could be started. The case emphasizes the importance of suspecting, investigating and energetically treating uncommon causes such as acute leukemia when an unusually severe clinical picture in a postpartum setting suggests such a possibility. This may prove to be life saving, particularly if the leukemia happens to be APL, a cancer with a very high cure rate.

Atypical presentation of acute promyelocytic leukaemia

Atypical presentation of acute promyelocytic leukaemia

Subdural Hemorrhage in a Child with Acute Promyelocytic Leukemia Presenting as Subtle Headache

Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML) and is rare in children (< 10% of childhood AML). It tends to bleed with disseminated intravascular coagulation (DIC) and intracranial hemorrhage complication is often fatal. We report a 12-year-old child with APL who suffered a subdural hemorrhage and initially presented with a subtle headache mistaken as the side effect of all-trans-retinoic acid (ATRA). Blood component therapy and a pediatric dosage of ATRA (25 mg/m2/day) combined with idarubicin as induction chemotherapy were administered in the first week, but the bleeding diathesis persisted and DIC profiles showed no improvement. The patient then developed photophobia, neck stiffness, and constant headache. Evidence of increased intracranial pressure (IICP) and persistent bleeding from previous venous puncture sites were also noticed clinically. DIC and life-threatening IICP were beyond control until the ATRA dosage was increased to adult levels (45 mg/m2/day). This case suggests that the ATRA dosage for pediatric APL patients must be modified according to clinical condition. Emergency brain imaging should be considered in APL patients with signs of IICP to distinguish intracranial lesions from ATRA complications.

Primary myeloid sarcoma of the testicle with t(15;17)

The first case of acute promyelocytic leukemia presenting as a solitary testicular mass (myeloid sarcoma) that relapsed in the contralateral testicle is described. The neoplastic cells strongly expressed chloroacetate esterase, myeloperoxidase, CD33, CD43, and weakly, CD117. The presence of many azurophil granules and Auer rods was detected by electron microscopy. Translocation (15;17)(q22;q21.1) was revealed by cytogenetics and was verified by fluorescence in situ hybridization. Contralateral testicle is a favorite site for recurrence in a subset of testicular myeloid sarcomas. Subclassification of all cases of myeloid sarcoma ought to be attempted.

Acute promyelocytic leukemia presenting as Fournier's syndrome in infancy

Fournier's syndrome (FS) is occassionaly encountered in surgical practice. We report a 4-month-old female with acute promyelocytic leukemia (APL) presenting as FS. This case is interesting in two aspects: the early onset of the APL and its presentation as FS, which is the first such case reported in the literature.

Multiple mechanisms: the example of vitamin A.

It has become evident that retinoids control differentiation, embryonal development, and tumorigenesis. In animal models, skin tumorigenesis has been shown to be prevented by retinoids, which in this organ function as antitumor promoters in the two-stage system using 7,12-dimethylbenz(a)anthracene (DMBA) as the initiator, and 12-tetradecanoyl-phorbol-13-acetate (TPA) as tumor promoter. Even though pharmacological doses applied topically appear to inhibit tumor formation, we found that papilloma and keratoacanthoma growth required physiological concentrations of retinoic acid and that vitamin A deficiency was even more effective than excess retinoid in inhibiting SENCAR mouse skin tumorigenesis. In human beings, oral administration of retinoic acid after tumor resection was effective in inhibiting the appearance of new tumors on the skin of four patients with Xeroderma Pigmentosum, and was effective in preventing new primary tumor formations in patients treated for head and neck cancer. The newly-discovered nuclear receptors for retinoic acid function as transcriptional activators for several genes. In patients with acute promyelocytic leukemia presenting with a reciprocal translocation of chromosome 17 to chromosome 15, the breakpoint has been identified in the retinoic acid receptor alpha gene, which forms a fusion gene with a new gene termed myl, on chromosome 15. Treatment of the patients with retinoic acid causes complete remission of the APL. It also appears to generate cells that do not bear the translocation. Therefore, retinoids may well function as modulators of carcinogenesis both at the promotion level as well as by causing differentiation of neoplastically transformed cells.

Acute promyelocytic leukemia presenting as a pelvic mass

The case history of a child with acute promyelocytic leukemia (APL) is reported to illustrate both an unusual presentation of APL as a pelvic mass and to review the pathophysiology and treatment of the disease. Therapy of APL consists of chemotherapy, namely adriamycin/daunomycin for remission induction, and of control of disseminated intravascular coagulation. A chloroma, if present, may require local irradiation in addition to chemotherapy. With aggressive management, the number of prolonged remissions may be greater for APL than for any other form of acute myelogenous leukemia (AML), with significant numbers of patients achieving five-year survival.

Acute promyelocytic leukaemia: a report of five cases with a comment on the diagnostic significance of serum vitamin B 12 determination.

Summary. Five patients with acute promyelocytic leukaemia presenting with initial leucopenia are reported. Severe and fatal bleeding complicated the course of the disease in four patients, associated with hypofibrinogenaemia in three of them. In addition, disseminated intravascular coagulation was found in three autopsied patients. An interesting and hitherto undescribed finding was the presence of high serum vitamin B, levels (2000=16000 pg/ml) found in all our patients in the absence of liver damage. In three of these patients an increased serum vitamin B12 binding capacity was observed (1500=6500 pg/ml). The diagnostic significance of these findings is discussed and it is suggested that the determination of serum vitamin 12 concentration and vitamin 12 binding proteins may be useful aids in the diagnosis of promyelocytic and myeloblastic leukaemia.