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

Bandyopadhyay, Sanjay; Bandyopadhyay, Debottam

doi:10.4103/0973-1482.77077

Cited by 6 publications

(9 citation statements)

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“…There are case reports of patients presenting with acute myocardial infarction and also ischemic stroke [7][8][9]. Rarely presentation may be with acute Budd-Chiari syndrome [10]. Presentation with thrombotic event portends a poor prognosis as was seen in our patient.…”

Section: Discussionmentioning

confidence: 55%

Acute Promyelocytic Leukemia Presenting as Ischemic Stroke in Young

Kapoor

Pati

Gupta

et al. 2012

Indian J Hematol Blood Transfus

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Coagulopathy in acute promyelocytic leukemia (APL) is a very complex disorder and is believed to result from interplay of various factors. Usually patients present with bleeding tendencies which can be life threatening. However, a few present with thromboses. Here, we report a young male with APL who presented with ischemic stroke in young. Case ReportA 23-year-old male presented to casualty with 1 month history of fever, symptomatic anemia followed, around 15 days later, by bleeding tendencies in the form of epistaxis, hematemesis, and melena. Two days prior to presentation to the hospital, he developed slurring of speech, pooling of secretions in mouth, and facial deviation toward the left side. His prior medical history was unremarkable with no history of hypertension, diabetes, smoking or alcohol consumption. On examination he was drowsy but arousable, disoriented to time, place, and person. He had pallor with widespread petechiae. His BP was 128/86 mmHg with temperature of 38.3°C. He was uncooperative for detailed neurological assessment; however, he had evidence of facial paresis on left side with extensor plantar on the left side. Spleen was palpable 4 cm below left costal margin. On investigation, hemoglobin (Hb) was 75 gm/l, total leukocyte count (TLC) was 85 9 10 9 /l, and platelets were 45 9 10 9 /l. Peripheral smear showed more than 90% abnormal hypergranular promyelocytes (Fig. 1). Blood chemistry was essentially normal. Coagulation profile revealed deranged prothrombin time (18 s with control of 12 s) with normal activated partial thromboplastin time (APTT), thrombin time (TT), and fibrinogen levels. D-Dimer levels were raised. Urgent noncontrast CT scan of the head was carried out which showed a hypodense area suggestive of infarct in the right parietooccipital region along with cerebral edema (Fig. 2). ECG showed sinus tachycardia with T wave inversion in anterior and lateral leads. Troponin (T) was negative. On the basis of peripheral smear findings and clinical presentation, the patient was immediately started on all-trans-retinoic acid (ATRA) at a dose of 45 mg/m 2 . As he presented in Sanz high risk category with very high TLC, ATRA was followed by parenteral daunorubicin at dose of 60 mg/m 2 . Blood component support with fresh frozen plasma, platelet concentrates, and packed RBC was given along with mannitol as the cerebral decongestant. Bone marrow examination done subsequently showed replacement by abnormal hypergranular promyelocytes with strong cytochemical positivity for myeloperoxidase (MPO) and sudan black (SBB) stains compatible with diagnosis of acute promyelocytic leukemia (APL) (Fig. 2). Immunophenotyping revealed positivity for CD 13, CD 33, CD 34, anti-MPO and negativity for HLA-DR, CD 2, CD 7, CD 79a, CD 117, and CD 19 in these cells. Coexpression of CD 10 was also noted. The diagnosis of APL was confirmed by positivity for PML-RARa (promyelocytic leukemia-retinoic acid receptor alpha) fusion transcript by reverse-transcription-polymerase chain reaction (RT-PCR). Howe...

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Section: Discussionmentioning

confidence: 55%

Acute Promyelocytic Leukemia Presenting as Ischemic Stroke in Young

Kapoor

Pati

Gupta

et al. 2012

Indian J Hematol Blood Transfus

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“…Myeloproliferative disorders are the most frequent haematological neoplasms associated with BCS and are diagnosed in 30–50% of cases. Other haematological neoplasms are rarely associated to BCS [3, 6, 8], and, to our knowledge, there are only 3 reported cases with simultaneous hepatic vein thrombosis and AML as initial presentation [9-11]. All these cases are related to acute promyelocytic leukaemia, which is known to turn patients more susceptible to thrombosis besides bleeding disorders [13].…”

Section: Discussionmentioning

confidence: 99%

“…Darwish Murad et al [8] identified the presence of one risk factor in 84% of the patients and two or more risk factors in 46%. Besides myeloproliferative disorders, which are commonly associated with BCS [3], other haematological neoplasms are quite rare and only published as case reports [9-11]. As malignancy is a formal and absolute contraindication for liver transplantation (LT) in the acute setting, its exclusion in the workup diagnosis of ALF is mandatory [2].…”

Section: Introductionmentioning

confidence: 99%

Budd-Chiari Syndrome and Acute Liver Failure: An Uncommon Presentation of Acute Myeloid Leukaemia

Costa

Freitas

Raposo

et al. 2020

GE Port J Gastroenterol

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Acute liver failure (ALF) is a rare entity, particularly in the context of Budd-Chiari syndrome (BCS). BCS is an uncommon disorder with multiple risk factors, most commonly myeloproliferative disorders. In BCS, active search and exclusion of underlying malignancy is mandatory, particularly in the context of ALF, as it may contraindicate liver transplantation (LT). We present the case of a healthy 29-year-old male, without known risk factors for liver disease, who presented to the emergency department with abdominal pain, ascites, and jaundice. BCS with consequent severe acute liver injury with rapid progression to ALF was diagnosed. The patient was listed for LT. The study of peripheral blood finally revealed myeloid blasts, and flow cytometry showed a population of blast cells with abnormal immunophenotypic profile (CD33+ and myeloperoxidase, MPO+). The bone marrow biopsy showed morphological and immunophenotypic aspects of acute myeloid leukaemia (AML) FAB M1. This diagnosis was considered a formal contraindication to LT, so the patient was delisted. ALF contraindicated rescue chemotherapy and AML contraindicated LT. The patient died 48 h after ICU admission. The search for underlying neoplasia is mandatory in the context of BCS, moreover with associated ALF, as it may limit lifesaving treatments and interventions to supportive and palliative care.

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“…43 PVT and acute BCS have been reported in patients with APL. 46,47 APL is associated with coagulopathy and both thrombotic and hemorrhagic complications, related to properties of the malignant promyelocytes which exert procoagulant as well as fibrinolytic and proteolytic activity. 48…”

Section: Svt In Other Hematological Malignanciesmentioning

confidence: 99%

Cancer-Associated Splanchnic Vein Thrombosis

Cohen

Caiano

Tufano

et al. 2021

Semin Thromb Hemost

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Splanchnic vein thrombosis (SVT), which includes portal, mesenteric, and splenic vein thrombosis and the Budd–Chiari syndrome, is an infrequent manifestation of venous thromboembolism (VTE). Like typical site VTE, SVT is also frequently associated with cancer, particularly intra-abdominal solid malignancies and myeloproliferative neoplasms (MPNs). The clinical presentation of SVT is nonspecific. Symptoms may be related to the underlying malignancy, and thrombosis is incidentally diagnosed by imaging studies for cancer staging or follow-up in a substantial proportion of cases. The occurrence of SVT predicts worse prognosis in patients with liver or pancreatic cancer and, not uncommonly, SVT may precede the diagnosis of cancer. Therefore, the occurrence of an apparently unprovoked SVT should prompt careful patient evaluation for the presence of an underlying malignancy or MPN. Cancer patients carry a high risk of VTE extension and recurrence and long-term anticoagulant treatment is suggested in the absence of high risk of bleeding. Either LMWH or direct oral anticoagulants (DOACs) are suggested for the treatment of patients with cancer-related SVT, although limited experience is available on the use of DOACs in these settings. Vitamin K antagonists (VKAs) are suggested for the short and long-term treatment of SVT associated with MPN. This review outlines the epidemiological aspects, pathogenesis, risk factors, and diagnosis of cancer-associated SVT, and addresses questions regarding the management of this challenging condition.

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

Cited by 6 publications

References 0 publications

Acute Promyelocytic Leukemia Presenting as Ischemic Stroke in Young

Acute Promyelocytic Leukemia Presenting as Ischemic Stroke in Young

Budd-Chiari Syndrome and Acute Liver Failure: An Uncommon Presentation of Acute Myeloid Leukaemia

Cancer-Associated Splanchnic Vein Thrombosis

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