Gastroenterology Research, ISSN 1918-2805 print, 1918-2813 online, Open Access
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Case Report

Volume 15, Number 3, June 2022, pages 148-154

Budd-Chiari Syndrome as an Initial Presentation of Non-Promyelocytic Acute Myelogenous Leukemia


Figure 1.
Figure 1. Image of skin changes associated with blue toe syndrome in this patient.
Figure 2.
Figure 2. (a) Peripheral smear showed large number of blast cells. (b) bone marrow shows bone marrow replaced by sheets of blasts consistent with acute myeloid leukemia.
Figure 3.
Figure 3. Ultrasound vascular Doppler image shows no hepatic vein outflow and sluggish but hepatopedal portal flow.
Figure 4.
Figure 4. Coronal (a) and axial (b) CT abdomen images show no hepatic vein outflow with mottled inhomogeneous enhancement of the liver. CT: computed tomography.
Figure 5.
Figure 5. Axial CT abdomen image shows caudate hypertrophy with hyperenhancement. CT: computed tomography.


Table 1. Laboratory Studies Done During Hospitalization
Test resultValueReference range
Hb: hemoglobin; WBC: white blood cell; PT: prothrombin time; aPTT: activated partial thromboplastin time; INR: international normalized ratio; LDH: lactate dehydrogenase; ALP: alkaline phosphatase; AST: aspartate aminotransferase; ALT: alanine aminotransferase; CMV: cytomegalovirus; EBV: Epstein-Barr virus; HSV: human herpes virus; PCR: polymerase chain reaction; BUN: blood urea nitrogen.
Hb, g/L5.712.8 - 16.4
Platelets, /µL6,000150,000 - 400,000
WBC, /µL17,4004,200 - 10,200
Blasts, %73%-
PT, s> 9911.7 - 14.5
aPTT, s> 12523.2 - 34.1
INR> 100.8 - 1
Fibrinogen, mg/dL< 15208 - 475
LDH, U/L> 4,00087 - 241
Total bilirubin, mg/dL8.40.2 - 1.0
ALP, U/L12445 - 117
AST, U/L7,15315 - 37
ALT, U/L2,99016 - 61
Albumin, g/dL3.63.4 - 5.0
Viral hepatitis panelNegative-
Lactic acid250.4 - 2.0
BUN, mg/dL327 - 18
Creatinine, mg/dL3.740.7 - 1.3
Blood culturesNegative
Protein C, %8070 - 120
Protein S, %11070 - 120
Antithrombin III, %12070 - 120
Lupus anticoagulantNegative
Anticardiolipin antibodiesNegative
Anti-phospholipid antibodiesNegative
Factor V LeidenNegative
Prothrombin mutation G20210ANegative


Table 2. Summary of Patients With Budd-Chiari Syndrome in Association With AML
AuthorCase numberAge (years)SexPresenting symptomsGeographic regionBCS as an initial presentationType of AML (FAB classification)TreatmentOutcome
AML: acute myeloid leukemia; FAB: French-American-British; ATRA: all-trans retinoic acid; rt-TPA: recombinant-type tissue plasminogen activator; LMWH: low molecular weight heparin.
Chillar et al, 1981 [9]129FemaleRestlessness, dehydration, delirium, jaundiceUSAYesAcute promyelocytic leukemia (M3)Deteriorated before treatment initiationDied
Riccio el al, 1989 [14]138FemaleWeakness, fatigue, coffee ground emesis, feverUSAYesAcute promyelocytic leukemia (M3V: microgranular variant)Deteriorated before treatment initiationDied
Assouline et al, 1995 [19]132MaleAbdominal pain and distension, weight gainFranceNoAcute promyelocytic leukemia (M3)ATRA, daunorubicine, cytosine arabinoside, systemic thrombolysis rt-TPA followed by heparinAlive
Kurt et al, 2005 [12]168MaleRight upper quadrant painUSANoAML (M4)Idarubicin and cytosine, arabinoside (treatment refractory)Not available
Amitrano et al, 2006 [7]127MaleEdema, epigastric pain, abdominal distensionItlayYesAMLDaunoblastine and cytarabine for 7 daysDied
Bandyopadhyay et al, 2010 [8]114MaleAbdominal distension, fatigue, fever, dark urineIndiaYesAcute promyelocytic leukemia (M3)Deteriorated before treatment initiationDied
Kayal et al, 2011 [11]13FemaleAbdominal distension, edemaIndiaYesAcute promyelocytic leukemia (M3)ATRA, daunomycin dalteparin, dexamethasoneDied
Havelange et al, 2014 [10]123MaleAbdominal distension, fatigue, fever, nauseaBelgiumYesAcute promyelocytic leukemia (M3)Not availableNot available
Natasa et al, 2014 [13]149FemaleHeadacheIranNoAcute promyelocytic leukemia (M3)ATRA, dexamethasone, idarubicin, LMWHAlive
Costa et al, 2021 [5]129MaleAbdominal pain and distension, fatigue, jaundicePortugalYesAML (M1)Deteriorated before treatment initiationDied
Kothadia et al, 2022 (present case)119MaleJaundice, fatigue and right upper quadrant abdominal painUSAYesAMLDeteriorated before treatment initiationDied