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Hematology week 1 > autoimmune diseases related to hematology > Flashcards

autoimmune diseases related to hematology Flashcards

1
Q

a consequence of long standing gastritis leading to atrophy of all the cells of the stomach (secretory).

A

Pernicious Anaemia

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2
Q

Autoimmune gastritis (AIG) is an organ-specific inflammatory
disorder leading

A

gastric atrophy & pernicious anemia.

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3
Q

Pathogenesis of AIG: caused by () with specificity for the () of the parietal cell proton pump () located in parietal cells of gastric mucosa. Gastric T cell recognition of the peptide epitopes results in secretion of ()

A

CD4+ T cells
beta sub-unit
(H+ /K+ ATPase)
TH1 cytokines.

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4
Q

Inflammation of gastric mucosa results in loss of parietal cell function 4 points

A

1- hydrochloric acid & intrinsic factor secretion;
2- iron deficiency,
3- vitamin B12 deficiency (some casespernicious anaemia);
4- gastric atrophy.

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5
Q

Pernicious Anaemia (PA) AutoimmuneFeatures

A

1- Anti–parietal cell antibodies
2- Anti–intrinsic factor antibodies

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6
Q

Anti–parietal cell antibodies

A

react with gastric parietal cells and are present in >90% of patients

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7
Q

major antigen for α-Parietal cell Abs

A

The acid-producing enzyme H+/K+-ATPase (92-kDa protein) present on the luminal membrane of parietal cells

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8
Q

occur in the serum, saliva, and gastric juice of 50-75% of patients with PA.

A

Anti–intrinsic factor antibodies

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9
Q

Pernicious Anemia is more common in individuals with

A

other autoimmune disease, especially polyglandular autoimmune syndromes, (e.g., Hashimoto’s thyroiditis, Graves’ disease, vitiligo, diabetes mellitus & Addison’s Disease).

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10
Q

acquired disorder characterized by isolated thrombocytopenia due to pathogenic anti-platelet antibodies

A

Immune Thrombocytopenia (ITP)

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11
Q

Acute ITP

A

usually self-limited occurring 1-3 weeks after a viral infection (e.g., nonspecific upper respiratory or gastrointestinal tract, rubella or chickenpox). Abrupt onset of bruising petechiae &/or epistaxis in previously healthy children is characteristic.

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12
Q

Chronic ITP

A

↑ incidence in females. Presenting symptoms include mucocutaneous bleeding, with menorrhagia, recurrent epistaxis & easy bruising.

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13
Q

mainly due to IgG autoantibodies which bind to platelets and megakaryocytes (MKs), targeting very abundant surface Ags such as glycoprotein (GP) αIIbβ3 (GPIIb/IIIa) and GPIb−IX.

A

Itp

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14
Q

Platelets with bound autoantibodies are subsequently recognized by () -> enhanced Ab-mediated platelet phagocytosis & removal from circulation ()

A

phagocytes bearing FcγRs
by reticuloendothelial cells (mainly in spleen).

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15
Q

Direct lysis of platelets by () has been detected along with an ↑ () cytokines & ↓ (_)

A

cytotoxic T lymphocytes (CTLs)
TH1/TH17
Tregs

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16
Q

Autoantibody binding to MKs & CTLs can lead to

A

deficiency in platelet release.

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17
Q

Many drugs can induce acute thrombocytopenia including

A

analgesics, antibiotics & sedatives.

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18
Q

Platelets: Common mechanism involves Ig binding () by their Fab (Ag-binding) region. () bind the Fc portion of the Ig. This may result in (_)

A

a platelet membrane Ag or Ag-drug combination
M⍬ Fc receptors
platelet removal & thrombocytopenia

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19
Q

Heparin-Induced Thrombocytopenia is mediated

A

by FcγRIIa

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20
Q

IgG autoantibodies that recognize PF4 in complex with heparin

A

FcRIIa.

21
Q

Ig Fc domains within the immune complex bind to the platelet receptor FcγRIIa, and activate platelets leading to

A

platelet aggregation (thrombosis) & clearance

22
Q

Rare disorder characterized by premature RBC destruction and anemia caused by autoantibodies that bind the RBC surface with or without complement activation

A

Autoimmune Hemolytic Anemia (AIHA)

23
Q

the severity of Autoimmune Hemolytic Anemia (AIHA) depends on:

A

 Characteristics of AutoAb (titer, ability to react at 37oC, ability to activate complement, and specificity & affinity for the autoAg).
 Ag characteristics (density on RBCs, immunogenicity)
 Patient related factors (age, B.M. compensation ability, M⍬ functionality, complement function/regulation)

24
Q

erythrocytes are destroyed in the blood vessel itself

A

Intravascular Hemolysis

25
Q

occurs in the hepatic & splenic macrophages within the
reticuloendothelial system

A

Extravascular Hemolysis

26
Q

in AIHA Autoantibodies my arise due to:

A

 immune dysregulation & loss of
immune tolerance
 exposure to an Ag similar to an autoAg  B lymphocyte neoplasm
 unknown reason (idiopathic).

27
Q

what can influence the development of autoAbs

A

The amount, type, duration of Ag exposure, in addition to genetic & environmental factors

28
Q

AIHA may be divided into four major categories:

A

Warm AIHA
Cold agglutinin disease
Paroxysmal cold hemoglobinuria
Mixed-type AIHA

29
Q

the most common type of AIHA comprising up to 70% of AIHA cases.

A

Warm Autoimmune Hemolytic Anemia (WAIHA)

30
Q

Most of these Abs are of IgG class, rarely IgA or IgM

A

Warm Autoimmune Hemolytic Anemia (WAIHA)

31
Q

Secondary WAIHA could be in conditions such as:

A

 Lymphoproliferative diseases
 Non-lymphoid neoplasms
 Autoimmune disorders Immunodeficiency disorders
 Viral infections

32
Q

comprises approximately 1⁄4 of AIHA cases.

A

Cold Agglutinin Disease (CAD)

33
Q

autoAb IgM that react optimally at 4oC.

A

CAD

34
Q

nonpathogenic cold agglutinins

A

polyclonal, occur in low titers & have no reactivity > 30oC.
(healthy individuals)

35
Q

Pathogenic cold agglutinins are

A

monoclonal, occur in high titers &
are capable of reacting at temperatures > 30oC -> can induce CAD.

36
Q

chronic cad

A

idiopathic or secondary to lymphoproliferative neoplasms

37
Q

acute CAD

A

occurring secondary to Mycoplasma pneumoniae infections, infectious mononucleosis or other viral infections

38
Q

IgM autoAb bind RBCs after exposure to cold

A

In CAD,

39
Q

The immune hemolysis is entirely complement-dependent.

A

CAD

40
Q

an acute form of cold-reactive hemolytic anemia.

A

Paroxysmal Cold Hemoglobinuria (PCH)

41
Q

most commonly seen in young children after respiratory viral infections & has childhood incidence as high as 32- 40% of children with autoimmune hemolytic anemia (5 yr median). rare in adults

A

Secondary PCH

42
Q

Paroxysmal Cold Hemoglobinuria (PCH) mechanism

A

Anti-P autoAb can bind to P Ag on RBCs & partially activate complement (C1-C4) at cold temperatures & upon warming to 37oC, full complement activation (C3 through C9) and hemolysis occur

43
Q

special feature of PCH

A

Exposure to cold temperatures is NOT required for hemolytic manifestations.

44
Q

what is mainly associated with SLE & lymphoma.

A

Mixed-Type AIHA

45
Q

Patients simultaneously develop IgG autoAb with optimum reactivity at 37oC (WAIHA) and IgM autoAb that react optimally at 0-10oC but have thermal amplitude of >30oC (CAD).

A

Mixed-Type AIHA

46
Q

Mixed-Type AIHA Hemolysis results from

A

a combination of extravascular & intravascular mechanisms.

47
Q

The warm autoAb are typically () with unclear specificity & the cold-reacting autoAb usually have ()specificity.

A

panreactive
anti-I

48
Q

which type of AIHA presents with a course that appears to be chronic with intermittent episodes of severe anemia.

A

Mixed-Type AIHA

Hematology week 1 (5 decks)

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