The disases associated with treatment in children with aplastic anemia

The disases associated with treatment in children with aplastic anemia Poster No.: C-2908 Congress: ECR 2010 Type: Scientific Exhibit Topic: Pe...
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The disases associated with treatment in children with aplastic anemia Poster No.:

C-2908

Congress:

ECR 2010

Type:

Scientific Exhibit

Topic:

Pediatric

Authors:

Y.-W. Kim , I.-O. Kim , W. S. Kim , H. J. Kim ; Yangsan/KR,

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Seoul/KR, Pusan/KR

Keywords:

Aplastic anemia, Treatment, Complication, CT, MRI

DOI:

10.1594/ecr2010/C-2908

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Purpose The Aplastic anemia is a blood disease, characterized by pancytopenia on peripheral blood, caused by decreased or deficient production of all hematopoietic progenitors. Despite the successful recovery of children with aplastic anemia following BM transplants, additional patients have had benefit form immune suppression therapy, predominantly antithymus globulin. The oral anabolic steroids have been helpful in a large residual pool of patients who have not able to benefit from these modalities. Many complications were caused by these treatments. We were to evaluate associated complications and their radiologic findings.

Methods and Materials 66 patients with Aplastic anemia were reviewed retrospectively. We reviewed the medical and radiologic records. •66 patients with Aplastic anemia ; retrospectively review ; medical & radiologic records ; pts with complication (10/66) •Radiologic review in complicated pts (n = 10) - Chest PA ( 3/10) - US ( 7/10) - CT (10/10) - MRI ( 4/10) - Angio & TAE ( 2/10)

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Results The complications were pneumonia, pleural effusion, nodular opacities in lung, abscesses, hepatic adenoma, iliopsoas hematoma, and peliosis hepatic in abdomen, and meningoencephalitis, intracranial hemorrhage (SDH, ICH) in brain. •

1. lung (3/10)

- nodular opacities - pleural effusion - bronchopneumonia •

2. Abdomen (8/10)

- liver & spleen abscess - hepatic adenoma (3) - iliopsoas hematoma - peliosis hepatis (3) •

3. brain (3/10)

- meningoencephalitis - SDH & ICH (Cbll & occipital) - cortical infract & ICH

#Hepatic Adenoma (Fig. 1 & 2) • •

a rare primary benign tumor Cause - androgen steroid

- glycogen storage dis (type 1) - liver adenomatosis •

Complication - hemorrhage

- malignant transformation

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a well-circumscribed, nonlobulated lesion

;solitary (80%), multiple •

Large subcapsular vessels / fat

No bile ductules, PV, HV •

R/F

1) US - hyperechoic appearance (d/t lipid, hemorrhage) - inhomogeneous (hemorrhage/ peliosis) 2) CDUS - large subcapsular vessels (flat continuous waveform) 3) CT - hypodense & calcification (1/2) on NCECT - nearly isointense on NCE, PVP, & DP images - variable CE (centripetal pattern) 4) MRI - heterogeneous high SI on T1WI - high SI on T2WI - subcapsular feeding vessels (early AP)

#Peliosis Hepatis • •

uncommon condition characterized by multiple blood-filled cavities mostly involving liver rare complication of androgen therapy

- hepatic dysfunction - hepatomegaly & elevated bilirubin levels •

Dynamic CT & MRI (Fig. 3 & 4)

- nodular areas in the center (AP) - centrifugal progression (PP) - diffuse homogeneous hyperintensity (DP)

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- no mass effect •

MRI (Fig. 4)

- multiple foci of increased SI on T2WI - variable SI on T1WI (subacute blood) - peripheral CE •

Hepatic angiography

- small ill-defined collections of contrast , persisted into the venous phase •

Confirm ; biopsy -> angiography -> dynamic CE

#Hemorrhage •

Intracranial hemorrahge (Fig. 5)

- a fatal complication - cause ; thrombocytopenia ; multifocal hemorrhagic tendency - ICH (3/6), SAH (2/6), SDH (1/6) •

Hemorrhagic infarction

- cause ; anabolic steroid therapy - at the time of hematologic improvement •

Hemorrhage in other site (Fig. 6)

- iliopsoas muscle

#Infections (Fig. 7) •

Lung

- pneumonia (bacterial, fungal) - pleural effusion

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Fungal infection

- sinusitis • • •

Hemorrhagic cystitis Liver and splenic abscess Meningoencephalitis

Images for this section:

Fig. 1: The hepatc adenomal in a child with aplastic anemia -- heterogenous hepatic masses with some calcifications show the delayed contrast enhancemet.

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Fig. 2: The ruptured adenoma of liver -- the surface discontinuity of hepatic adenoma and peritumoral hematoma are noted on liver CT images. The active extravasation was confirmed by hepatic angiogram.

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Fig. 3: Peliosis hepatis in a child of aplastic anemia during anaboric steroid treatment. The multiple low attenuated nodules are noted in right lobe liver. These lesions show centripetal contrast enhancement on delayed image.

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Fig. 4: The liver T2W(A) AND T1WI(B) show multiple foci of heterogenous SIs in liver. These small lesions reveal delayed filling-in of contrast on dynamic CE images.

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Fig. 5: Intracranial Hemorrhage in a patient with aplastic anemia during steroid.

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Fig. 6: The 9 years-old boy with aplastic anemia complaint abdominal pain. There is hematoma in right psoas muscle on US scan.

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Fig. 7: The liver and splenic abscess in a patient with aplastic anemia treatment. -- low attenuated nodule in liver and spleen with peripheral calcifiation. -- target-like contrast enhancement of these nodules

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Conclusion The knowledge of these various complications can be helpful in the diagnosis and management in children with aplastic anemia.

References

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Hepatocellular Adenoma: Multiphasic CT and Histopathologic Findings in 25 Patients. Tomoaki Ichikawa, MD, Michael P. Federle, MD, Luigi Grazioli, MD and Michael Nalesnik, MD, Radiology. 2000;214:861-868. Liver Adenomatosis: Clinical, Histopathologic, and Imaging Findings in 15 Patients. Luigi Grazioli, MD, Michael P. Federle, MD, Tomoaki Ichikawa, MD, Eric Balzano, MD, Michael Nalesnik, MD and Juan Madariaga, MD, Radiology. 2000;216:395-402.) Hepatic Adenomas: Imaging and Pathologic Findings. Luigi Grazioli, MD, Michael P. Federle, MD, Giuseppe Brancatelli, MD, Tomoaki Ichikawa, MD, Lucio Olivetti, MD and Arye Blachar, MD. Radiographics. 2001;21:877-892.) Cerebral venous thrombosis associated with tentorial subdural hematoma during oxymetholone therapy. Kon Chu, Dong-Wha Kang, Dong-Eog Kim, Jae-Kyu Roh, Journal of the Neurological Sciences 185 (2001). 27-30. Images in clinical medicine. Peliosis hepatis.Walter E, Möckel J. N Engl J Med. 1997 Nov 27;337(22):1603. Peliosis hepatis: diagnosis by magnification wedged hepatic venography. J Lyon, JJ Bookstein, CA Cartwright, A Romano and DJ Heeney, Radiology, Vol 150, 647-649 Peliosis hepatitis. Pliskin M. Radiology. 1975 Jan;114(1):29-30. Hepatocellular adenoma: MR imaging features with pathologic correlation KY Chung, WW Mayo-Smith, S Saini, A Rahmouni, M Golli, and D Mathieu, AJR 1995;165:303-308 Splenic and hepatic peliosis: MR findings. CK Maves, KH Caron, GS Bisset, 3d, and R Agarwal. AJR 1992;158:75-76

Personal Information Department of Radiology, College of Medicine, Pusan National Univeristy, Children's Hospital, Yangsan, Korea

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[email protected]

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