PATHOLOGY SGD 4:

DISEASES-OF-THE-IMMUNE-SYSTEM

GROUP-4
 CASE #1

Your patient is a 45-year-old man admitted due to a vehicular

accident with severe trauma. You order a blood transfusion with 2
units packed RBC, properly typed and crossmatched. However, a
clerical error resulted in the wrong type of blood transfused.
Immediately after transfusion, the patient becomes faint and
hypotensive. Work up reveals free hemoglobin in the plasma and
urine.
 1. WHAT HAPPENED TO THIS PATIENT?

When a person gets wrong blood type, the immune system begins attacking the
blood related to ABO/Rh incompatibility leading to increase in body temperature,
sudden decrease in blood pressure causing faintness.

Transfusion with the wrong blood type can cause a severe reaction that may be life-
threatening. If you have many blood transfusions, you are more likely to have problems
from immune system reactions. A reaction causes your body to form antibodies that
attack the new blood cells.
 2. WHAT TYPE OF HYPERSENSITIVITY REACTION IS
INVOLVED?
Type II hypersensitive reactions involve antibody-mediated destruction of cells. This type of reaction is best
seen by blood-transfusion reactions, in which host antibodies react with foreign antigens on the incompatible
transfused blood cells and mediate destruction of these cells. Type II hypersensitivity is typified by a
transfusion reaction in which mismatched red blood cells are rapidly destroyed by specific preformed antibodies
(anti-ABO or -Rh) and complement.

An antibody mediated process in which IgG and IgM antibodies are directed against antigens on cells (such
as circulating red blood cells) or extracellular material (such as basement membrane). This subsequently leads
to cell lysis, tissue damage or loss of function through mechanisms such as complement activation via the
classical complement pathway Antibody-dependent cellular cytotoxicity or anti-receptor activity. The activation
of the complement system results in opsonization, the agglutination of red blood cells, cell lysis, and cell death.
These reactions usually take between 2 and 24 hours to develop.
 3. GIVE A SHORT DISCUSSION OF THIS TYPE OF
HYPERSENSITIVITY REACTION. GIVE OTHER EXAMPLES.

• Type II hypersensitivity reaction involve antibody- mediated destruction of cells. It is also called as
cytotoxic hypersensitivity reaction.

• This type is best exemplified by blood transfusion reactions, in which host antibodies reacts with
foreign antigens on the incompatible transfused blood cells and mediated destruction of these cells.

• Type II hypersensitivity reaction results when IgG or IgM binds to cell surface

• Activating complement and binding Fc receptors on Tc cells promoting ADCC both processes result in
lysis of the Ab- coated cell.

• Type II hypersensitivity is typified by a transfusion reaction in which mismatched red blood cells are
rapidly destroyed by specific preformed antibodies (anti-ABO or -Rh) and complement.
 • Type II hypersensitivity is due to antibody reactivity against cell membrane-associated antigens that
results in cytolysis.

• Type II reaction is produced by mismatched blood types which destroys foreign RBC by complement-
mediated lysis triggered by IgG.

• Which in result produces fever, intravascular clots, lower back pain and Hgb in urine .

EXAMPLES:

• Drug intake in patients with drug-induced lupus. In this type, anti-red blood cell or anti-dsDNA
antibodies are produced as a result of a drug attaching to red blood cells resulting in drug-induced
systemic lupus erythematosus (SLE).

• Autoimmune hemolytic anemia, leukopenia, thrombocytopenia

 4. GIVE SHORT SUMMARIES OF OTHER TYPES OF
HYPERSENSITIVITY REACTIONS. GIVE 1-2 EXAMPLES OF
EACH.

Hypersensitivity

Hypersensitivity (also called hypersensitivity reaction or intolerance)

refers to undesirable reactions produced by the normal immune
system, including allergies and autoimmunity.
 CASE #2

Working as an internist in a hospital, a 34-year-old woman is referred to

you by her primary care physician. The patient has been suffering from
several months of feeling tired, with bouts of fever and joint pain. Your
physical exam shows a female not in cardiopulmonary distress but with a
red, flat rash on the bridge of her nose and on both cheeks. She also has
multiple raised, erythematous patches on the skin, and ulcers on her
palate. You decide to do laboratory tests for this patient.
 1. BASED ON YOUR PRIMARY CONSIDERATION, WHAT
LABORATORY TESTS WILL YOU ORDER FOR THIS PATIENT?

Antinuclear antibody titer is the primary laboratory test used to

diagnose systemic lupus erythematosus. Anti-Nuclear Antibody (ANA)
Test.

Anti-nuclear antibodies (ANA) are autoantibodies to the nuclei of

your cells. 98% of all people with systemic lupus have a positive ANA
test, making it the most sensitive diagnostic test for confirming diagnosis
of the disease. Blood test also be done.
 Some other also be done. Such as
 CBC (Complete Blood Count) with differential
 Serum creatinine
 Urinalysis with microscopy
 ESR (Erythrocyte Sedimentation Rate) or CPR level
 Complement levels
 Liver function tests
 Creatine kinase assay
 Spot protein/spot creatine ratio
ANA TEST
 2. WHAT WILL BE OTHER THINGS YOU NEED TO LOOK FOR
IN THIS PATIENT?

As the patient was suffering from tiredness, bouts of fever, red flat
rash on cheeks, ulcers and erythematous patches on skin and the things
we have to check and look for anxiety, blood in urine, depression,
sensitivity of light, Raynaud’s syndrome, scaly rashes or butterfly rashes
on skin, poor circulation in fingers and toes, pneumonia, pulmonary
emboli, immune complexes in blood and inflammation of fibrous sac and
severe abdominal pain in heart.
3) GIVE THE CRITERIA FOR THE DIAGNOSIS OF
THIS DISEASE?
 4. EXPLAIN THE PATHOPHYSIOLOGY OF THIS DISEASE.

In SLE, biopsies of affected skin show deposition of Ig at the dermal-

epidermal junction (DEJ), injury to basal keratinocytes, and inflammation
dominated by T lymphocytes in the DEJ and around blood vessels and dermal
appendages. Clinically unaffected skin may also show Ig deposition at the DEJ.
These patterns are not specific for dermatologic SLE; however, they are highly
suggestive. In renal biopsies, the pattern and severity of injury are important in
diagnosis and in selecting the best therapy. Most recent clinical studies of lupus
nephritis have used the International Society of Nephrology (ISN) and the Renal
Pathology Society (RPS) classification. In the ISN/RPS classification, the
addition of “a” for active and “c” for chronic changes gives physicians
information regarding the potential reversibility of disease
 The system focuses on glomerular disease, although the presence of tubular interstitial and
vascular disease, as well as the chronicity score in both glomeruli and interstitium, are important in
predicting clinical outcomes. In general, class III and IV disease, as well as class V accompanied by
III or IV disease, should be treated with aggressive immunosuppression if possible, because there is a
high risk for end-stage renal disease (ESRD) if patients are untreated or undertreated.

In contrast, treatment for lupus nephritis is not recommended in patients with class I or II disease
or with extensive irreversible changes. In the recent Systemic Lupus International Collaborating
Clinic (SLICC) criteria for classification of SLE, a diagnosis can be established on the basis of renal
histology in the presence of lupus auto antibodies, without meeting additional criteria .Histologic
abnormalities in blood vessels may also determine therapy. Patterns of vasculitis are not specific for
SLE but may indicate active disease: leukocytoclastic vasculitis is most common. Lymph node
biopsies are usually performed to rule out infection or malignancies. In SLE, they show nonspecific
diffuse chronic inflammation.
 CASE # 3

A 25-year-old male call center agent is seen at your clinic for a chief
complaint of “fatigue.” The patient tells you that he has been
experiencing a few months’ worth of tiredness and generalized weakness,
with unexplained weight loss. The patient admits to a history of having
intercourse with multiple partners, both male and female. Some of these
partners are sex workers. You order an STD panel of lab tests and
unfortunately, both the screening test and confirmatory test for HIV are
positive.
1. HOW DOES HIV INFECT AND CAUSE IMMUNODEFICIENCY?
(EXPLAIN THE PATHOGENESIS OF HIV)

Acquired immunodeficiency syndrome (AIDS) is a chronic, potentially life-

threatening condition caused by the human immunodeficiency virus (HIV). By
damaging your immune system, HIV interferes with your body's ability to fight
infection and disease. Immunosuppression due to HIV infection is a resultant of
several factors including defective innate signaling pathways, increased viral
replication and virus load, gradual loss of peripheral CD4+T cells and depletion of T
lymphocytes at mucosal sites that collectively lead to progressive immune deficiency
and AIDS.HIV infection is caused by the human immunodeficiency virus. You can
get HIV from contact with infected blood, semen, or vaginal fluids.
 2. IF THE PATIENT GOES UNTREATED, WHAT ARE THE
POSSIBLE OUTCOMES FOR THIS PATIENT?

 HIV is one of the most deadliest diseases till date. Although there is no known cure for
HIV, the progression can be slowed down using medications.

 HIV stands for human immunodeficiency virus, as the name suggests it attacks the
immune system of the body, to be specific, white blood cells which have CD4
markers(CD4T cells).

 Because it attacks the immune system of the body, tendency to fight off infection is
drastically reduced and the patient is prone to multiple opportunistic diseases at the same
time. This condition is called Acquired Immunodeficiency Syndrome commonly referred
as AIDS.
 Though it takes 8 to 10 years for HIV to completely develop into AIDS, early symptoms
may be found within 3 to 5 years of infection.

 When HIV is left untreated it turns into AIDS. When a patient is suffering from AIDS
their immune system is so compromised that it can’t even fight off minor bacterial
infection.

 Multiple opportunistic diseases like Pneumocystis pneumonia, Tuberculosis,

Cytomegalovirus, Toxoplasmosis can infect the patient due to their weak immune system.

 In some cases a tumour is developed which leads to cancer even with no family history
of the disease. Lymphoma and Kaposi’s sarcoma are common type of cancer seen in patients with
AIDS.
 GROUP MEMBERS:

1. ADDULA DEEPAK REDDY 11. SANA PRANAV PRADEEP

2. DARSI NAGA VENKATA YUVRAJ 12. SANNAYELA SIVA KUMAR
3. DHARAVATH SHIVLAL NAYAK 13. SYED AMAN ANSARY
4. GAUTAM SHRISTI 14. THIRUNAVUKKARASU HARINI
5. GORASYA MONIKA 15. YALLAMAVARI MAHESHWARI
6. ISMAIL GASIM 16. YARAMALA JOHN MOSES
7. JASMINE JENA
8. KOTHAPALLI DRAKSHAYANI
9. PETLURU TEJASWINI
10. VELAMURU SAI LAKSHMI
PRIYANKA
THANK YOU