Clinical Pathological Conference Dr. David Gonzales May 5, 2006 - PowerPoint PPT Presentation

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Clinical Pathological Conference Dr. David Gonzales May 5, 2006

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Title: Clinical Pathological Conference Dr. David Gonzales May 5, 2006

1
Clinical Pathological Conference Dr. David
Gonzales May 5, 2006
2

CC Abdominal pain x 2 months
PMH 49 y/o AAM with h/o abdominal pain x 2
months.
Pain is epigastric, constant, severe, disturbs
sleep, no radiation, worse with meals and
associated with early satiety.
Associated with nausea, had emesis x 2 two months
ago.
He reports black stools for 2 weeks but denies
BRBPR.
He has had poor PO intake and reports losing gt 20
lbs over 2 months.
He also c/o constipation.
No abdominal distention. No jaundice or acholic
stools.
He denied fever or chills but did say he felt
hot and had some sweats.

He went to the ER at another facility 2 months
ago and was given an unknown medication after X
rays and sonogram of the abdomen were done
reportedly negative.
His pain worsened and he developed back pain
radiating down both lower extremities. He
returned to the ER a few weeks later and was
given hydrocodone/APAP and a PPI and asked to f/u
with a PCP.
His PCP continued the PPI but pain persisted. He
then developed a new R flank pain, pain radiating
down R LE worsened and he complained of R LE
weakness.
He went back to the ER at the other hospital and
after further workup was transferred to our
hospital.

PMHx No PCP
H/o MVA x 2 with R elbow .
PSHx none
Allergies NKDA
Medications
Pantoprazole 40 mg po qd
Hydrocodone/APAP as needed
Social History Single. Lives with his parents.
Will not disclose sexual orientation but has not
been sexually active for gt 1 year, no h/o STDs.
Occasional tobacco-cigars for 3 years, occasional
ETOH, denies recreational drug use.
Family History Mother with DM, HTN Father with
HTN
ROS Positive for fatigue, weakness, feeling
hot, sweats and weight loss as above. Also
reports hiccups and R LE weakness , otherwise
negative.

PHYSICAL EXAM
Gen emaciated 49 y/o AA male
BP 138/92. HR 111, RR 18, Wt 68.5 kg, T-afebrile
HEENT Normocephalic, atraumatic, PERRL, EOMI, OP
clear, slightly dry.
Neck- Supple. No JVD, bruit, LAD, thyromegaly.
Lungs CTAB
Cv RRR. S1S2, no murmur, gallop, rub.
Tachycardia
Abd Periumbilical and RUQ fullness, Prominent
veins above the umbilicus, NABS. Liver edge
palpable, firm, nontender-span 11 cms. Ill
defined mass about 8x8 cm palpable in the
periumbilical area, lower right border rounded,
other borders poorly defined, firm, non tender.
No splenomegaly. No shifting dullness.
Rectal- Normal sphincter tone. Brown, guaiac ve
stool
Neuro CNS II-XII grossly intact, DTRs 2/4,,
sensation intact, F-to-N intact. Strength 5/5
b/l UEs, RLE prox 3/5, distal 4/5, LLE prox 4/5,
distal 4/5. Gait not evaluated

6
Labs

8.3 \ 8.9 / 540
/ 27.1 \
N82, L8, M6,
E1,Bands1, Metamyelocytes 2
Fe lt20 ug/dL, ferritin 316 ng/ml, transferrin
171mg/dL, satlt 8.
MCV was 78, his stool occult blood was initially
negative but turned positive 3 days later. RDW
14.9
129 98 23 / 73 Ca 8.6 mg/dL
4.6 24 1.7 \
Tp 8.1 g/dL Alb 3.4 g/dL Glob 3.7g/dL
AST 78 U/dL ALT 79 u/dL AlkP 686 u/dL Tbili 0.6
mg/dL
SPEP-protein 6.8-high Alpha 1 globulin 0.32, low
albumin
FENAlt 1 UA-wnl
Lipase 1391 U/L, amylase 302 U/L, Uric acid 14.6
mg/dL

More tests were ordered..

Fecal H. pylori Antigen -ve
HIV status ve CD4-absolute 110, - 29
The creatinine and sodium normalized with
hydration
The alkaline phosphatase dropped to the 190s
later.

8
Radiology

CXR Small to moderate left pleural effusion with
left basilar atelectasis.
CT Spine- Disc disease L5-S1-no fracture.
Other imaging was obtained..

9
CT Abdomen/Pelvis

Massive retroperitoneal adenopathy, Moderate
R
hydronephrosis, Prominent gastric
folds/thickened gastric
wall.

10
A diagnostic procedure was performed.
11
CPC

David Gonzales
Presbyterian Hospital of Dallas
May 5, 2006

12
Case presentation

49 year old male with epigastric pain x 2 months
Constant, severe
Early satiety
20 pound weight loss, sweats, feeling hot

13
Management of dyspepsia

Unintended weight loss
Persistent vomiting
Dysphagia/Odynophagia
Anemia
Hematemesis
Palpable mass
iron deficiency anemia
Family history of upper gastrointestinal cancer
Previous gastric surgery
Jaundice

14
Case presentation

Sonogram (-)
No improvement with PPI
Pain extends to right flank and unilateral lower
extremity weakness develops
Remainder of history not incredibly helpful

15
Differential Diagnosis

Tumor
Lymphoma
Biliary or other sites in GI tract
Infection with atypical organism
Gastritis/gastropathy

16
Physical Exam

Mild tachycardia
Emaciated
Periumbilical/RUQ fullness with firm, 8cm mass
liver palpable
Right leg 3/5 prox, 4/5 distal left leg 4/5
sensation and reflexes normal

17
Labs

Microcytic anemia
Ferritin 316 (28-365)
Ironlt20, transferrin 171, 8 saturation
Mild thrombocytosis also argues for iron
deficiency
Mild hyponatremia and renal insufficiency which
corrected with hydration

18
Labs

Alkaline Phosphatase about 5 x normal with normal
bilirubin
Mild transaminitis
Lipase and amylase elevated
Ca 19-9 moderately elevated
LDH and uric acid very elevated

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SPEP

A screening test for plasma cell disorders
A clone secretes a homogenous (M for monoclonal)
protein
If M protein is found, it must be characterized
by immunofixation
This patient had high alpha-1 globulin and low
albumin

21
And hes got AIDS

CD 4 110
Abdominal pain is common in HIV
Opportunistic infection
Regular stuff (gastritis, cholecystitis, etc)
Medication related
Malignancy

22
55 yo AA male with AIDS and

Abdominal pain with fever, weight loss, and
sweats
Abdominal mass
Iron-deficiency anemia
Markers of high cell turnover
Pancreaticobiliary abnormalities

23
Imaging

CXR small left pleural effusion with
atelectasis
CT Abdomen/Pelvis
Massive retroperitoneal adenopathy
Moderate R hydronephrosis
Prominent gastric folds
No info on liver, pancreas, or kidney assume
normal

24
Retroperitoneal Adenopathy

Retroperitoneal fibrosis
Testicular Cancer
Renal Cell Carcinoma
Opportunistic infection
Lymphoma

25
Enlarged Gastric Folds

Menetriers disease
Foveolar hyperplasia in the body and fundus of
the stomach
Symptoms include pain, asthenia, anorexia
Often have hypoalbuminemia secondary to
protein-losing enteropathy

26
Enlarged Gastric folds

Zollinger-Ellison (gastrinoma)
High gastrin output causes acid hypersecretion ?
ulcers, primarily in the duodenum and distally
Often associated with diarrhea (3/4 of pts)
Weight loss only present in 17
MEN 1

27
Enlarged Gastric Folds

H. Pylori-associated gastritis
Anisakiasis
Adenocarcinoma
Lymphoma
GI vs. HIV-related

28
Gastrointestinal lymphoma

Defined as localized disease in the GI tract or
presentation predominantly in the GI tract
Role of MALT
Spread from adjacent nodes
Diffuse large B-cell lymphoma also seen

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HIV and malignancy

Increased incidence similar to transplant
recipients
Length and degree of immunosuppression likely
play roles
Role of HIV and other viruses including EBV

31
HIV and lymphoma

Risk increases directly as CD4 count drops
3 General types
Primary CNS lymphoma
Primary effusion lymphoma
Non-Hodgkins lymphoma

32
HIV and systemic lymphoma

Tend to be aggressive
High proliferation
Spontaneous cell death
2 main histologic types
Diffuse large B cell
Burkitts or Burkitts-like

33
Burkitts lymphoma

3 types
Endemic (African)
Non-endemic (American)
Immunodeficient
Translocation of C-myc is important

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To summarize

55 year old male with AIDS
Abdominal pain from a large, fast-growing
retroperitoneal mass
B symptoms (probably)
Evidence of high cell turnover (uric acid and LDH
LE motor weakness and R hydronephrosis
Suspect nerve root and ureteral compression from
adenopathy

38
Summary

Diagnostic test lymph node biopsy
EUS?

39
Diagnosis

AIDS-associated NHL, favor Burkitts
Rapid presentation
High cell turnover
Rare

40
M.C.

Diffuse high grade B-cell lymphoma,
Burkitt- like morphology
2/1/06 Retroperitoneal core biopsy
2/1/06 Bone marrow biopsy
2/2/06 Duodenal mass biopsy
Gastric mass biopsy
2/2/06 CSF cytology

41
M.C.

Pathology
Diffuse infiltrate B-cells (CD20)
Uniform intermediate size nuclei
Fine chromatin, nucleoli present
Basophilic cytoplasm with vacuoles
Numerous mitoses, admixed histiocytes

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M.C.

Immunophenotype
Positive CD20, CD79, CD10, Bcl-6, CD38
Negative CD34, CD117, CD3, Tdt, Bcl-2,
CD138, CD5, CD23, CD56
Light chain restriction Kappa
Ki-67 proliferative index high (gt 90)

48
M.C.

Diagnosis
Diffuse high grade B-cell lymphoma, Burkitt-like
morphology
Definitive diagnosis pending FISH studies for
c-myc translocation
c-myc FISH results
1st lab negative
2nd lab positive

49
FISH
LSI IGH/MYC, CEP 8
LSI MYC break apart
50
Classic Burkitts lymphoma

Morphology
Diffuse, monomorphic cells
Interspersed macrophages starry sky
Neoplastic nuclei are uniform, round intermediate
size
Multiple (2-5) small distinct nucleoli uniform
Moderate amount basophophilic vacuolated
cytoplasm
Frequent mitoses, apoptotic bodies

51
Classic Burkitts lymphoma

Immunophenotype
B-cell CD20, CD19, CD22
Surface monoclonal Ig
Positive CD10, CD43
Negative TdT, CD34, Bcl-2, CD138

52
Burkitt Lymphoma (BL)

Rare in non-immune depressed
lt 1 of NHL
HIV () 1000 fold incidence of BL

53
Lymphoma in HIV/AIDS

NHL in 4-10 AIDS patients
Relative risk for NHL 60-200 fold
10 of all NHL in USA
NHL affects all AIDS groups equally
HL relative risk 10 fold in AIDS

54
Lymphoma in HIV/AIDS

HIV virus is not directly lymphomagenic
Deficient immune surveillance/cellular immunity
HIV associated infections
Activation/proliferation B-cells
B-cell genetic instability mutations,
deletions, translocation of tumor suppressor
genes/oncogenes
Clonal expansion of genetically altered B-cells
B-cell NHL

55
Lymphoma in HIV/AIDS