Transcript AIDS-related lymphoma

IN THE NAME OF GOD
CASE PRESENTATION
HISTORY
• A 31 years Old female from Chatroud kerman
with complaints of cough, moderate
hemoptysis, was hospitalized for 3 days. The
patient complained of weakness and fatigue
earlier than a year ago. Than a few months
ago with sparseness palpitations, chest
pain,mild dyspnea. Patient also complained of
a dry mouth, sore tongue ,weight loss 15kg
over last year,. Patient had recently mild
dysphagia to solids intermitant.
past history
• she did not have any previous history of
known illness, except admission for
lobectomy<right parotid>becase swelling
parotid years ago.patologyreport:
1-intra parotid lymphoid tissue with dailated
duct .
2-lymph node,post auricular resection reactive
hyperplasia.
Drug history
 1-tab ferrus sulfate daily for 3 month ago.
 2-tab folic acid 1mg daily.
 3-tab alprazolam 0/25mg QHS perevious
10day.
 4-tab propranolol 20mgBID.
Family History
• There was no significant point.Her husband
died due to seizures, 8 months ago and SHe
was living with her 12 year old daughter.
• Personal and Social History:
-She was not smoker and opium addict.
PHYSICAL EXAMINATION.
• Patient is ayoung age female, awake and
oriented, with distress, she was thin and
pale.
• Vital Sign:
PR:100 RR:22 BP:90/60 AxilaryT:38
PHYSICAL EXAMINATION
• The conjectiva is paile-Trachea and thyroid
were normal- No adenopathy.
• Chest examination:
• decrease of sound in basal of left side and
fine Crackle in 1/3 lower left side.heart exam
was normal.
• Abdomen: There was no distention,
tenderness, hepatomegaly, splenomegaly and
ascites.
• Upper and lower extremitie was normal.
LABORATORY TEST
CBC was 2 months ago:
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WBC:4000
RBC:4300000
HG:10.8
HCT:34.7
 MCV:80
 MCH:25.1
 MCHC:31.3
 Plat:108,000
LABORATORY TEST
BMA was normal
 ECHOCARDIOGRAPGY:
 EF:60 %
 PAP:40
mild pericardial effiusion.
LABORATORY TEST
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Normal range
C3:90
70-196
C4:17
10-40
CH50:100 101-300
AntidsDNA:9.4(<25)
ANA:
NEGATIVE
PANCA: NORMAL
AntiGBM:13.9 (<25)
CANCA: NORMAL
LABORATORY TEST
 CBC:
 WBC:5400
 RBC:4,180,000
 HG:10.5
 PLT:143,000
 HCT:33.9
 MCV:81.1
 MCH:25.1
 MCHC:31
Biochemistry
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BS:100
Na:146
K:4.7
Urea:51
Creat:1.4
Ca:4.2
ESR:73
U/A:NORMAL
AST:72
ALT:53
ALP:147
Bil Total:0.4
Bil Diect:0.2
 PT:16
 PTT:30
 INR:1.4
CHEST CTSCAN
• a mass lesion in the middle mediastinum with
is seen that for better evaluation ct scan with
contrast is recommended.wall thickening of
esophagus is seen that endoscopy is
recommended. Left plural effiusion is vesible.
ABDOMINOPELVICE SONOGRAPHY
• Liver, biliary tract, pancreas and urinary
tract Were normal. Size spleen 140mm
and mild plural effiusion in left
hemithorax
Problem list
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Moderate hemoptysis
Cough& dyspnea
Fever
Chest pain
Palpitation
Intermitant dysphagia
weight loss
weakness and fatigue
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Bicytopenia
Pap:40 & mild p.e
ESR:73
AST:72 & ALT:53
Middle mediastinal
mass
 Mild P.E
mediastinum
The mediastinum extends from the thoracic
inlet to the diaphragm, and contains many
vital structures, including the heart and great
vessels, and esophagus.
anterior mediastinum
• The anterior compartment (also referred to as
the
anterosuperior
compartment
or
retrosternal space) is anterior to the
pericardium and includes the thymus, the
extrapericardial aorta and its branches, the
great veins, and lymphatic tissue.
middle mediastinum
• The middle compartment is bounded by the
pericardium
anteriorly,
the
posterior
pericardial reflection, the diaphragm, and the
thoracic inlet. This compartment includes the
heart,
intrapericardial
great
vessels,
pericardium, and trachea.
posterior mediastinum
• The posterior compartment extends from the
posterior pericardial reflection to the
posterior border of the vertebral bodies and
from the first rib to the diaphragm. It includes
the esophagus, vagus nerves, thoracic duct,
sympathetic chain, and azygous venous
system.
masse in anterior compartment
• Thymic tumors (thymomas), thymic carcinomas,
thymic carcinoid tumors, and thymolipomas), thymic
cysts.
• germ cell tumors, Hodgkin and non-Hodgkin
lymphomas.
• intrathoracic goiter, thyroid tumors, parathyroid
adenomas.
• connective tissue tumors (eg, lipomas, liposarcomas,
lymphangiomas,
lymphangiohemangiomas,
hemangiomas), and pericardial cyst.
Masses of the middle
mediastinum
• thyroid
tumor,
goiter,
tracheal
tumors,
aortopulmonary paraganglioma (chemodectoma),
bronchogenic cysts and lymphomas.
• A middle mediastinal mass may also represent
lymphadenopathy as a result of infectious, malignant
(metastatic), and idiopathic (eg, sarcoidosis)
etiologies.
• Thymic masses and pericardial cysts have been
reported in the middle mediastinum, however, these
lesions generally occur in the anterior mediastinum
posterior mediastinum mass
• neurogenic tumors , esophageal tumors and
duplication cysts, hiatus hernia, and
neurenteric cysts.
• Extramedullary hematopoiesis, pancreatic
pseudocyst, and achalasia have all been
reported as unusual causes of a posterior
mediastinal mass.
• Aneurysms of the aorta may occupy any
compartment depending upon their anterior,
intrapericardial, or posterior location.
Clinical manifestations
Airway compression can lead to recurrent pulmonary
infection and/or hemoptysis
• Esophageal compression can cause dysphagia
• Involvement of the spinal column can result in paralysis
• Phrenic nerve damage can present with an elevated
hemidiaphragm
• Hoarseness can occur due to recurrent laryngeal nerve
involvement
• Horner's and superior vena cava syndromes arise due to
sympathetic ganglion and superior vena caval involvement,
respectively
Clinical manifestations
Evaluation mediastinal mass
Evaluation mediastinal mass
BRONCOSCOPy
Abnormal mucosa,external pressure and a
small masslike lesion in the entrance of
LMB&bleeding from LMB.
Bleeding controlled with transhexamic
acid&BAL and biopsy was performed.
• Thoracic surgery was consulted, because of
stress and lack of patient consent were not
candidates for mediastinoscopy.
diagnosis
The hemoptysis was controlled after the
patient re-bronchoscopic and appropriate
biopsy was performed.
HIV testing was requested.
Pathology report : NHL(DLBCL).
HIV test:posetive.
Lymphoproliferative disorders in
immunocompromised patient
1- acquired immune deficiency syndrome (AIDS)related lymphoma (ARL):
(a) usual lymphomas primarily involving the lung,
DLBCL and Burkitt lymphoma being the commonest
(b) others: primary effusion lymphoma (PEL),
plasmablastic lymphoma
2- post-transplantation lymphoproliferative disorder
(PTLD), which includes reactive processes, neoplasms
and lymphoid proliferations of uncertain
nature.
AIDS-related lymphoma
• Infection with HIV predisposes to the
development of neoplasms, including
lymphoma.
• AIDS-related lymphoma is generally divided
into three types:
 systemic non-Hodgkin lymphoma (NHL)
primary central nervous system lymphoma.
 primary effusion ("body cavity") lymphomas
AIDS-related lymphoma
Patients infected with human immunodeficiency
virus (HIV) have a higher risk to develop non
Hodgkin’s lymphoma (NHL) with high frequency of
extranodal lesions. Lymphoma occurs in 3 to 10%
of patients with AIDS and is the second most
common malignancy in this group
AIDS-related lymphoma
• This neoplasm is usually of high-grade B-cell type
and the most frequent location is in the CNS, GI
system, liver, spleen, and bone marrow. Thoracic
involvement occurs in < 10% of the cases.
• A variety of clinical forms of AIDS-related
lymphoma in the chest x-ray have been
described, including pleural effusion, interstitial
and alveolar lung disease, peripheral nodules,
and occasionally, hilar and mediastinal
adenopathies.
AIDS-related lymphoma
• HIV-associated lymphoma is most commonly
diagnosed in patients with advanced HIV, a
low CD4 count (often <100/microL), high HIV
viral load, and a prior diagnosis of AIDS .
• Since the introduction of highly antiretroviral
therapy, the incidence of HIV-associated
lymphoma has declined and the median CD4
count at diagnosis has increased.
AIDS-related lymphoma
• The risk of developing NHL in the setting of
HIV increases directly with the level of
immune system dysfunction. The incidence,
pathology, clinical presentation and course of
AIDS-related lymphomas do not seem to be
related to the risk factor for the HIV infection
itself .
• Epstein-Barr virus (EBV) coinfection is a risk
factor for and involved in the pathogenesis of
subtypes of NHL.
AIDS-related lymphoma
• systemic lymphoma in the HIV-positive
population is characterized by frequent B
symptoms (ie, fever, weight loss, night
sweats), extranodal disease, involvement of
unusual locations.
TREATMEN
• Survival without treatment in patients with
aggressive NHL, such as DLBCL, can be
measured in months. With the advent of
combination
chemotherapy
with
cyclophosphamide, doxorubicin, vincristine,
and prednisone (CHOP).
• Survival has been further improved with the
addition of rituximab to standard CHOP-based
therapy (R-CHOP).