World J Oncol

World Journal of Oncology, ISSN 1920-4531 print, 1920-454X online, Open Access

Article copyright, the authors; Journal compilation copyright, World J Oncol and Elmer Press Inc

Journal website http://www.wjon.org

Case Report

Volume 4, Number 2, April 2013, pages 95-101

ALK+ Anaplastic Large Cell Lymphoma With Bladder Involvement Presenting as Fever of Unknown Origin: A Case Report and Literature Review

Figure 1. Contrast-enhanced CT scan of the abdomen and pelvis reveals intrabdominal and retroperitoneal adenopathy of uncertain origin. Adenopathy includes right retrocrural, retroperitoneal, celiac, peripancreatic, mesenteric, iliac and pelvic lymph nodes. Upper retroperitoneum reveals confluent nodal mass measuring 37.3 millimeters in image b.

Figure 2. Periaortic lymph node biopsy reveals a mature and polymorphous lymphoid population with some macrophages and normal histologic architecture. There is an intact capsule and no lymphocyte migration outside of the capsule. There are follicles with germinal centers and normal interfollicular cellularity. Photos a, b and c represent low, medium and high power views, respectively.

Figure 3. Bladder biopsy sections reveal a dense infiltrate composed of large abnormal lymphoid cells at low and medium power views. Photo a shows diffuse, ragged infiltration of bladder muscle by cells with dark nuclei and moderate size. An example of these cells is represented in photo b. Immunohistochemical stains showed that these large cells are positive for CD45, CD3, CD5, CD4, and CD30. CD30 positive cells are represented in photo c. Occasional large cells are also positive for ALK-1. They are negative for CD20, CD8, CD34, Tdt, CD56, pankeratin, synaptophysin and chromogranin. Findings are consistent with an ALK+ anaplastic large cell lymphoma.

**Table 1.** Diagnostic Laboratory Work-Up Performed at Initial Hospital Admission
	Lab	Result		Lab	Result
Wnl: within normal limits; Neg: negative. ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; C3/C4: complement; RF: rheumatoid factor; LDH: lactate dehydrogenase; ANA: anti-nuclear antigen; dsDNA: double stranded DNA; SS-a/b: Sjogren’s syndrome; Scl: scleroderma; RNP: ribonucleoprotein; Sm: Smith; C/P-ANCA: cytoplasmic/perinuclear antineutrophil cytoplasmic antibodies; TSH: thyroid stimulating hormone; T4: thyroid hormone; SPEP: serum protein electrophoresis; AFP: alpha-fetoprotein; B-HCG: beta-human chorionic gonadotropin; PPD: purified protein derivative; ASO: anti-streptolysin titer; Ig: immunoglobulin; PCR: polymerase chain reaction; EBV: Ebstein-barr virus; HIV: human immunodeficiency virus.
Inflammatory	ESR	65 (H)	Infectious	PPD	Neg
	CRP	12.9 (H)		ASO	Neg
	C3	132		Hepatitis panel	Neg
	C4	30		Legionella urinary antigen	Neg
	RF	< 20		Parvovirus IgG	Wnl
	LDH	315 (H)		Parvovirus IgM
Antibodies	ANA	+ 1:160		Coccidiodes antibody	Neg
	dsDNA	Wnl		CMV IgG	Wnl
	SS-a/SS-b	Neg		CMV IgM	Wnl
	Scl-70	Neg		CMV PCR	Undetectable
	RNP	Neg		EBV IgG	5.05 (H)
	Sm	Neg		EBV IgM	0.01
	Sm/RNP	Neg		EBV PCR	Undetectable
	C-ANCA/P-ANCA	< 1:20		Lyme Disease PCR	Undetectable
Endocrine	TSH	1.42		Malarial smear	Neg
	Free T4	1.8		HIV screen	Neg
	A.M.-Cortisol	27		Histoplasmosis urinary antigen	Neg
	SPEP	No monoclonal protein		Blastomycosis urinary antigen	Neg
	AFP	1.7		Blood cultures × 6	No growth
	B-HCG	1.7		Urine cultures × 2	No growth

Table 1. Diagnostic Laboratory Work-Up Performed at Initial Hospital Admission

Lab

Result

Lab

Result

Wnl: within normal limits; Neg: negative. ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; C3/C4: complement; RF: rheumatoid factor; LDH: lactate dehydrogenase; ANA: anti-nuclear antigen; dsDNA: double stranded DNA; SS-a/b: Sjogren’s syndrome; Scl: scleroderma; RNP: ribonucleoprotein; Sm: Smith; C/P-ANCA: cytoplasmic/perinuclear antineutrophil cytoplasmic antibodies; TSH: thyroid stimulating hormone; T4: thyroid hormone; SPEP: serum protein electrophoresis; AFP: alpha-fetoprotein; B-HCG: beta-human chorionic gonadotropin; PPD: purified protein derivative; ASO: anti-streptolysin titer; Ig: immunoglobulin; PCR: polymerase chain reaction; EBV: Ebstein-barr virus; HIV: human immunodeficiency virus.

Inflammatory

ESR

65 (H)

Infectious

PPD

Neg

CRP

12.9 (H)

ASO

Neg

132

Hepatitis panel

Neg

Legionella urinary antigen

Neg

< 20

Parvovirus IgG

Wnl

LDH

315 (H)

Parvovirus IgM

Antibodies

ANA

+ 1:160

Coccidiodes antibody

Neg

dsDNA

Wnl

CMV IgG

Wnl

SS-a/SS-b

Neg

CMV IgM

Wnl

Scl-70

Neg

CMV PCR

Undetectable

RNP

Neg

EBV IgG

5.05 (H)

Neg

EBV IgM

0.01

Sm/RNP

Neg

EBV PCR

Undetectable

C-ANCA/P-ANCA

< 1:20

Lyme Disease PCR

Undetectable

Endocrine

TSH

1.42

Malarial smear

Neg

Free T4

1.8

HIV screen

Neg

A.M.-Cortisol

Histoplasmosis urinary antigen

Neg

SPEP

No monoclonal protein

Blastomycosis urinary antigen

Neg

AFP

1.7

Blood cultures × 6

No growth

B-HCG

1.7

Urine cultures × 2

No growth

**Table 2.** Diagnostic Imaging Performed at Initial Hospital Admission
Image	Result
Neg: negative; LVSF: left ventricular systolic function; EF: ejection fraction; LVH: left ventricular hypertrophy; MR: mitral regurgitation; TR: tricuspid regurgitation; RVSP: right ventricular systolic pressure; HTN: hypertension.
CT chest w/IV contrast	Neg. for mass or lymphadenopathy. Lungs clear.
Renal Ultrasound	Bilateral complex renal cysts: 2.9 cm in right kidney and 1.8 cm in the left kidney.
Transthoracic Echocardiogram	- Normal cardiac chamber size. Normal LVSF, EF 60%. Mild concentric LVH. - Trivial MR. Mild TR, estimated RVSP of 42 mmHg suggesting mild pulmonary HTN. - Small circumferential pericardial effusion w/o evidence of tamponade.
Nuclear Medicine Bone Scan	Nonspecific uptake in the right superolateral bony orbit.
Bilateral Lower Extremity Venous Doppler	Negative for deep venous thrombosis.

Table 2. Diagnostic Imaging Performed at Initial Hospital Admission

Image

Result

Neg: negative; LVSF: left ventricular systolic function; EF: ejection fraction; LVH: left ventricular hypertrophy; MR: mitral regurgitation; TR: tricuspid regurgitation; RVSP: right ventricular systolic pressure; HTN: hypertension.

CT chest w/IV contrast

Neg. for mass or lymphadenopathy. Lungs clear.

Renal Ultrasound

Bilateral complex renal cysts: 2.9 cm in right kidney and 1.8 cm in the left kidney.

Transthoracic Echocardiogram

- Normal cardiac chamber size. Normal LVSF, EF 60%. Mild concentric LVH.
- Trivial MR. Mild TR, estimated RVSP of 42 mmHg suggesting mild pulmonary HTN.
- Small circumferential pericardial effusion w/o evidence of tamponade.

Nuclear Medicine Bone Scan

Nonspecific uptake in the right superolateral bony orbit.

Bilateral Lower Extremity Venous Doppler

Negative for deep venous thrombosis.