Gastroenterol Res

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

Article copyright, the authors; Journal compilation copyright, Gastroenterol Res and Elmer Press Inc

Journal website https://www.gastrores.org

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 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. Ultrasound vascular Doppler image shows no hepatic vein outflow and sluggish but hepatopedal portal flow.

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. Axial CT abdomen image shows caudate hypertrophy with hyperenhancement. CT: computed tomography.

**Table 1.** Laboratory Studies Done During Hospitalization
Test result	Value	Reference 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/L	5.7	12.8 - 16.4
Platelets, /µL	6,000	150,000 - 400,000
WBC, /µL	17,400	4,200 - 10,200
Blasts, %	73%	-
PT, s	> 99	11.7 - 14.5
aPTT, s	> 125	23.2 - 34.1
INR	> 10	0.8 - 1
Fibrinogen, mg/dL	< 15	208 - 475
LDH, U/L	> 4,000	87 - 241
Total bilirubin, mg/dL	8.4	0.2 - 1.0
ALP, U/L	124	45 - 117
AST, U/L	7,153	15 - 37
ALT, U/L	2,990	16 - 61
Albumin, g/dL	3.6	3.4 - 5.0
Viral hepatitis panel	Negative	-
CMV, HSV, EBV PCR	Negative
Lactic acid	25	0.4 - 2.0
BUN, mg/dL	32	7 - 18
Creatinine, mg/dL	3.74	0.7 - 1.3
Blood cultures	Negative
Protein C, %	80	70 - 120
Protein S, %	110	70 - 120
Antithrombin III, %	120	70 - 120
Lupus anticoagulant	Negative
Anticardiolipin antibodies	Negative
Anti-phospholipid antibodies	Negative
Factor V Leiden	Negative
Prothrombin mutation G20210A	Negative

Table 1. Laboratory Studies Done During Hospitalization

Test result

Value

Reference 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/L

5.7

12.8 - 16.4

Platelets, /µL

6,000

150,000 - 400,000

WBC, /µL

17,400

4,200 - 10,200

Blasts, %

73%

PT, s

> 99

11.7 - 14.5

aPTT, s

> 125

23.2 - 34.1

INR

> 10

0.8 - 1

Fibrinogen, mg/dL

< 15

208 - 475

LDH, U/L

> 4,000

87 - 241

Total bilirubin, mg/dL

8.4

0.2 - 1.0

ALP, U/L

124

45 - 117

AST, U/L

7,153

15 - 37

ALT, U/L

2,990

16 - 61

Albumin, g/dL

3.6

3.4 - 5.0

Viral hepatitis panel

Negative

CMV, HSV, EBV PCR

Negative

Lactic acid

0.4 - 2.0

BUN, mg/dL

7 - 18

Creatinine, mg/dL

3.74

0.7 - 1.3

Blood cultures

Negative

Protein C, %

70 - 120

Protein S, %

110

70 - 120

Antithrombin III, %

120

70 - 120

Lupus anticoagulant

Negative

Anticardiolipin antibodies

Negative

Anti-phospholipid antibodies

Negative

Factor V Leiden

Negative

Prothrombin mutation G20210A

Negative

**Table 2.** Summary of Patients With Budd-Chiari Syndrome in Association With AML
Author	Case number	Age (years)	Sex	Presenting symptoms	Geographic region	BCS as an initial presentation	Type of AML (FAB classification)	Treatment	Outcome
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]	1	29	Female	Restlessness, dehydration, delirium, jaundice	USA	Yes	Acute promyelocytic leukemia (M3)	Deteriorated before treatment initiation	Died
Riccio el al, 1989 [14]	1	38	Female	Weakness, fatigue, coffee ground emesis, fever	USA	Yes	Acute promyelocytic leukemia (M3V: microgranular variant)	Deteriorated before treatment initiation	Died
Assouline et al, 1995 [19]	1	32	Male	Abdominal pain and distension, weight gain	France	No	Acute promyelocytic leukemia (M3)	ATRA, daunorubicine, cytosine arabinoside, systemic thrombolysis rt-TPA followed by heparin	Alive
Kurt et al, 2005 [12]	1	68	Male	Right upper quadrant pain	USA	No	AML (M4)	Idarubicin and cytosine, arabinoside (treatment refractory)	Not available
Amitrano et al, 2006 [7]	1	27	Male	Edema, epigastric pain, abdominal distension	Itlay	Yes	AML	Daunoblastine and cytarabine for 7 days	Died
Bandyopadhyay et al, 2010 [8]	1	14	Male	Abdominal distension, fatigue, fever, dark urine	India	Yes	Acute promyelocytic leukemia (M3)	Deteriorated before treatment initiation	Died
Kayal et al, 2011 [11]	1	3	Female	Abdominal distension, edema	India	Yes	Acute promyelocytic leukemia (M3)	ATRA, daunomycin dalteparin, dexamethasone	Died
Havelange et al, 2014 [10]	1	23	Male	Abdominal distension, fatigue, fever, nausea	Belgium	Yes	Acute promyelocytic leukemia (M3)	Not available	Not available
Natasa et al, 2014 [13]	1	49	Female	Headache	Iran	No	Acute promyelocytic leukemia (M3)	ATRA, dexamethasone, idarubicin, LMWH	Alive
Costa et al, 2021 [5]	1	29	Male	Abdominal pain and distension, fatigue, jaundice	Portugal	Yes	AML (M1)	Deteriorated before treatment initiation	Died
Kothadia et al, 2022 (present case)	1	19	Male	Jaundice, fatigue and right upper quadrant abdominal pain	USA	Yes	AML	Deteriorated before treatment initiation	Died

Table 2. Summary of Patients With Budd-Chiari Syndrome in Association With AML

Author

Case number

Age (years)

Sex

Presenting symptoms

Geographic region

BCS as an initial presentation

Type of AML (FAB classification)

Treatment

Outcome

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]

Female

Restlessness, dehydration, delirium, jaundice

USA

Yes

Acute promyelocytic leukemia (M3)

Deteriorated before treatment initiation

Died

Riccio el al, 1989 [14]

Female

Weakness, fatigue, coffee ground emesis, fever

USA

Yes

Acute promyelocytic leukemia (M3V: microgranular variant)

Deteriorated before treatment initiation

Died

Assouline et al, 1995 [19]

Male

Abdominal pain and distension, weight gain

France

Acute promyelocytic leukemia (M3)

ATRA, daunorubicine, cytosine arabinoside, systemic thrombolysis rt-TPA followed by heparin

Alive

Kurt et al, 2005 [12]

Male

Right upper quadrant pain

USA

AML (M4)

Idarubicin and cytosine, arabinoside (treatment refractory)

Not available

Amitrano et al, 2006 [7]

Male

Edema, epigastric pain, abdominal distension

Itlay

Yes

AML

Daunoblastine and cytarabine for 7 days

Died

Bandyopadhyay et al, 2010 [8]

Male

Abdominal distension, fatigue, fever, dark urine

India

Yes

Acute promyelocytic leukemia (M3)

Deteriorated before treatment initiation

Died

Kayal et al, 2011 [11]

Female

Abdominal distension, edema

India

Yes

Acute promyelocytic leukemia (M3)

ATRA, daunomycin dalteparin, dexamethasone

Died

Havelange et al, 2014 [10]

Male

Abdominal distension, fatigue, fever, nausea

Belgium

Yes

Acute promyelocytic leukemia (M3)

Not available

Natasa et al, 2014 [13]

Female

Headache

Iran

Acute promyelocytic leukemia (M3)

ATRA, dexamethasone, idarubicin, LMWH

Alive

Costa et al, 2021 [5]

Male

Abdominal pain and distension, fatigue, jaundice

Portugal

Yes

AML (M1)

Deteriorated before treatment initiation

Died

Kothadia et al, 2022 (present case)

Male

Jaundice, fatigue and right upper quadrant abdominal pain

USA

Yes

AML

Deteriorated before treatment initiation

Died