Block 4 Final Flashcards
(238 cards)
1
Q
Describe the common findings in Wiskott Aldrich syndrome (labs)
A
Low IgM
High IgA & IgE
Normal IgG
Low T cell levels
2
Q
Low IgM
High IgA & IgE
Normal IgG
Low T cell levels
labs indicate which condition?
A
Wiskott Aldrich syndrome
3
Q
Describe the clinical signs of Wiskott-Aldridge syndrome
A
Thrombocytopenia
Eczema
Recurrent infections
Atopic dermatitis (Extensor side)
4
Q
ThrombocytopenIa
Eczema
Recurrent infections
What’s the condition?
A
Wiskott-Aldridge syndrome
5
Q
What causes Wiskott-Aldridge syndrome?
A
Mutation of the WASP protein (Xp11.23)
6
Q
Mutation of the WASP protein (Xp11.23) causes which condition?
A
Wiskott-Aldridge syndrome
7
Q
Describe the common findings in Isolated IgA deficiency
A
1) Very low serum & secretory IgA
2) High risk of respiratory tract allergies & autoimmune disease
3) Defective BAFF (Bcell activating cytokine)
8
Q
1) Very low serum & secretory IgA
2) High risk of respiratory tract allergies & autoimmune disease
3) Defective BAFF (Bcell activating cytokine)
Are all common findings of which condition?
A
Isolated IgA deficiency
9
Q
Describe the clinical features of Isolated IgA Deficiency
A
Recurrent sino-pulmonary infections & diarrhea
10
Q
Recurrent sino-pulmonary infections & diarrhea & low IgA indicate which conditions
A
Isolated IgA Deficiency
11
Q
What is a complication of Isolated IgA Deficiency
A
Type II severe anaphylaxis when given a blood transfusion
12
Q
Describe the common findings of DiGeorge syndrome
A
1) Thymic hypo/anaplasia (no Tcell immunity)
2) Hypocalcemia (no parathyroid)
13
Q
1) Thymic hypo/anaplasia (no Tcell immunity)
2) Hypocalcemia (no parathyroid)
are all common findings of which condition?
A
DiGeorge
14
Q
What is the cause of DiGeorge?
A
Deleted TBX1 region on 22q11 (causes 3rd & 4th pharyngeal pouches to not develop)
15
Q
Deleted TBX1 region on 22q11 (causes 3rd & 4th pharyngeal pouches to not develop)
A
DiGeorge
16
Q
Describe the clinical features of DiGeorge
A
1) Recurrent infections
2) Tetany
3) Congenital heart defects
4) Flat nasal bridge
5) Widened epicanthal fold
6) Low set/back rotated ears
7) small philtrum & chin
17
Q
1) Recurrent infections
2) Tetany
3) Congenital heart defects
4) Flat nasal bridge
5) Widened epicanthal fold
6) Low set/back rotated ears
7) small philtrum & chin
Are features of which condition?
A
DiGeorge
18
Q
What are the causes of X-linked Agammaglobinulemia?
A
Mutated Burton tyrosine kinase (Btk) on Xq21.22 (no Bcell development)
19
Q
Mutated Burton tyrosine kinase (Btk) on Xq21.22 (no Bcell development)
cause which condition?
A
causes of X-linked Agammaglobinulemia
20
Q
Describe the common findings (labs) in X-linked Agammaglobinulemia
A
1) None/Low B cell #
2) Low Igs (all of them)
3) CD19 +/ IgM - Bcells
4) Normal Tcells
5) Underdeveloped germinal centers in LN
21
Q
1) None/Low B cell #
2) Low Igs (all of them)
3) CD19 +/ IgM - Bcells
4) Normal Tcells
5) Underdeveloped germinal centers in LN
Are all common findings in which condition?
A
X-linked Agammaglobinulemia
22
Q
Describe the clinical features of X-linked Agammaglobinulemia
A
1) Recurrent respiratory infections
- Acute/chronic pharyngitis
- Sinusitis
- Bronchitis
- Osteitis media
- Pneumonia
2) Giardia lamblia (if IgA is low)
3) Recurrent diarrhea & GERD
23
Q
1) Recurrent respiratory infections
- Acute/chronic pharyngitis
- Sinusitis
- Bronchitis
- Osteitis media
- Pneumonia
2) Giardia lamblia (if IgA is low)
3) Recurrent diarrhea & GERD
Are features of which condition?
A
clinical features of X-linked Agammaglobinulemia
24
Q
Describe the etiology of X-linked Agammaglobinulemia
A
More common in males & it doesn’t become apparent until 6 months
25
What are the pathogens that pose the most serious threat to persons with X-linked Agammaglobinulemia?
H. influenza
S. pneumoniae
S. aureus
Enteroviruses (Echo/polio/coxsackie)
26
H. influenza
S. pneumoniae
S. aureus
Enteroviruses (Echo/polio/coxsackie)
Pose the most risk to persons with which autoimmune disorder?
X-linked Agammaglobinulemia
27
What is a consideration when vaccinating children with X-linked Agammaglobinulemia?
you can't give them live vaccines because they don't have B-cells to fight it!!!!
28
Describe the clinical features of SCID
1) Oral thrush
2) Extensive diaper rash
3) Failure to thrive
4) Morbilliform rash & GVHD (shortly after birth)
5) Recurrent infections
6) Chronic watery diarrhea (viral)
29
1) Oral thrush
2) Extensive diaper rash
3) Failure to thrive
4) Morbilliform rash & GVHD (shortly after birth)
5) Recurrent infections
6) Chronic watery diarrhea (viral)
Are all features of which condition?
SCID
30
Which pathogens pose the most risk to a person with SCID?
1) Candida albicans
2) Pneumonitis jiroveci (sand paper lung)
3) Pseudomonas
4) CMV
5) Vericella
31
1) Candida albicans
2) Pneumonitis jiroveci (sand paper lung)
3) Pseudomonas
4) CMV
5) Vericella
Are pathogens that pose the most risk to a person with which autoimmune condition?
SCID
32
Describe the cause of X-linked SCID (subtype)
Mutation in a common y-chain that messes with interleukin production especially IL-7 (lymphoid progenitors) & IL15 (NK proliferation)
33
Mutation in a common y-chain that messes with interleukin production especially IL-7 (lymphoid progenitors) & IL15 (NK proliferation)
IS THE CAUSE OF which AUTOIMMUNE disease?
cause of X-linked SCID (subtype
34
Describe the common findings in X-linked SCID
1) Normal neutrophil #
2) Black thymic shadow on X-ray
3) Low Igs
4) Low Tcell #
35
1) Normal neutrophil #
2) Black thymic shadow on X-ray
3) Low Igs
4) Low Tcell #
Are all common findings of which condition?
X-linked SCID
36
Describe the etiology of X-linked SCID
More common in young boys
37
Describe the cause of AUTO-REC SCID
Deficient ADA enzyme (Adenosine deaminase) causing an accumulation of deoxy-ATP which is toxic to lymphocytes
&
Mutated RAG gene (blocks B & T cell development)
38
Deficient ADA enzyme (Adenosine deaminase) causing an accumulation of deoxy-ATP which is toxic to lymphocytes
&
Mutated RAG gene (blocks B & T cell development)
The cause of which condition?
AUTO-REC SCID
39
Describe the etiology of Sjogren syndrome
Older females (50-60yrs) typically with thyroid issues
40
Describe the clinical features of Sjogren syndrome
1) Keratoconjunctivitis sicca (dry/burning/itchy eyes & blurred vision)
2. Xerostomia (dry mouth)
- diff swallowing solids
- tongue fissures
- Parotid gland enlargement
3. Extra glandular disease
- Synovitis
- Diffuse pulmonary fibrosis
- Peripheral neuropathy
41
1) Keratoconjunctivitis sicca (dry/burning/itchy eyes & blurred vision)
2. Xerostomia (dry mouth)
- diff swallowing solids
- tongue fissures
- Parotid gland enlargement
3. Extra glandular disease
- Synovitis
- Diffuse pulmonary fibrosis
- Peripheral neuropathy
All features of which condition?
Sjogren syndrome
42
Describe the immuno-markers of Sjogren syndrome?
1) Anti-CCLP ab **
2) IgM vs IgG
3) RA factor
4) SS-A (Ro)
5) SS-B (La)
43
In sjogren syndrome presenting with SS-A (Ro) is indicative of what prognosis?
poor prognosis (systemic spread of disease shows extra glandular symptoms)
44
What is the first form of Sjogren syndrome?
1) Sicca syndrome (primary) associated with defective:
- HLA-B8
- HLA-DR3
- DRW52
- HLA-DQA1
- HLA-DQB1
45
An autoimmune disorder associated with defective:
- HLA-B8
- HLA-DR3
- DRW52
- HLA-DQA1
- HLA-DQB1
Sicca syndrome (primary)
46
What is the second form of Sjogren syndrome?
2) legit when Sjogrens is associated with any other autoimmune disorder the most common being Rheumatoid arthritis
47
Describe the etiology of Systemic sclerosis (scleroderma)
Females 50-60yrs old usually
48
Describe the common findings in Systemic sclerosis (scleroderma)
Widespread damage of small blood vessels & progressive fibrosis in the interstitial & perivascular tissues of the skin, Gi, kidneys, heart, muscles, & lungs
49
Widespread damage of small blood vessels & progressive fibrosis in the interstitial & perivascular tissues of the skin, Gi, kidneys, heart, muscles, & lungs
Indicates which condition?
Systemic sclerosis (scleroderma)
50
Describe the clinical features of Limited Systemic Sclerosis
"CREST"
Calcification
Raynaud's
Esophageal dysmotility (rubber hose)
Sclerodactyly (claw hands)
Telangiectasia
Typically skin symptoms are limited to the forearms, face & fingers
51
"CREST"
Calcification
Raynaud's
Esophageal dysmotility (rubber hose)
Sclerodactyly (claw hands)
Telangiectasia
Typically skin symptoms are limited to the forearms, face & fingers
Are features of which condition?
Limited Systemic Sclerosis
52
Describe the cause of Limited Systemic Scleroderma
The body makes anti-SCL-70 (Targets DNA topoisomerase)
53
The body makes anti-SCL-70 (Targets DNA topoisomerase)
the cause of which condition?
Limited Systemic Sclerosis
54
In a persons with systemic scleroderma anti-centromere production indicates which prognosis?
It indicates CREST (aka limited SS) a good prognosis
55
In a persons with systemic scleroderma anti-RNA pol III production indicates which prognosis?
It indicates diffuse SS which has a poorer prognosis
56
Describe the common findings in Diffuse scleroderma
Widespread diffuse atrophy of the skin that spreads to the viscera of organs
57
Widespread diffuse atrophy of the skin that spreads to the viscera of organs
Indicated which condition?
Diffuse scleroderma
58
Describe the histological findings typical of Diffuse Scleroderma?
1) edema
2) Perivascular infiltration
3) Thickened basal lamina
59
1) edema
2) Perivascular infiltration
3) Thickened basal lamina
Are common histological findings in which condition?
Diffuse Scleroderma
60
Describe the clinical features of Diffuse scleroderma
1) Diffuse sclerotic atrophy of the skin
2) autoamputation (fingers)
3) Progressive atrophy and fibrosis of Gi & Esophagus (Esophageal dysmotility & Barrets)
4) Fibrosis of intralobular kidney arteries
5) Pulmonary hypertension/vasospasms
6) Interstitial fibrosis
7) Pericarditis with effusion
8) Myocardial fibrosis
61
1) Diffuse sclerotic atrophy of the skin
2) autoamputation (fingers)
3) Progressive atrophy and fibrosis of Gi & Esophagus (Esophageal dysmotility & Barrets)
4) Fibrosis of intralobular kidney arteries
5) Pulmonary hypertension/vasospasms
6) Interstitial fibrosis
7) Pericarditis with effusion
8) Myocardial fibrosis
Are all features of which condition?
Diffuse scleroderma
62
Describe the etiology of Acute myeloid leukemia (AML)
Happens at all ages but peaks at 60yrs
63
Describe the causes of Acute myeloid leukemia (AML)
AML gene disruptions due to:
1) t(8;21) translocation(disrupts RUNX1)
2) INV(16) (disrupts CBFB)
3) t(15;17) translocation(acute promyelocytic leukemia)
4) Benzene (leather industry)
64
AML gene disruptions due to:
1) t(8;21) translocation(disrupts RUNX1)
2) INV(16) (disrupts CBFB)
3) t(15;17) translocation(acute promyelocytic leukemia)
4) Benzene (leather industry)
Are all contributing factors to developing which condition?
Acute myeloid leukemia (AML)
65
Describe the common findings of Acute myeloid leukemia
1) There (20%) myeloblasts in the bone marrow
2) Neoplastic cells have delicate chromatin & azurophilic granules
66
1) There (20%) myeloblasts in the bone marrow
2) Neoplastic cells have delicate chromatin & azurophilic granules
What's the condition?
Acute myeloid leukemia
67
1) Auer rods
2) AML t(15;17) translocation
What's the condition?
Acute Promyelocytic leukemia
68
Describe the features of Acute myeloid leukemia
1) Recurrent infections'
2) Anemia
3) Thrombocytopenia (increased PT & PTT)
4) Neutropenia
69
1) Recurrent infections'
2) Anemia
3) Thrombocytopenia (increased PT & PTT)
4) Neutropenia
Are all features of which condition?
Acute myeloid leukemia
70
1) Cell differentiation
2) Fibrosis (increased Fibrogenic cytokines) (Downs syndrome)
3) Pseudopod formation***
What's the condition
AML Megakaryoblastic type
71
1) Myeloperoxidase +
2) CD33/34+
What's the condition
Acute Myeloblastic leukemia
72
1) Non-specific esterase +
2) Blueberry rash
What's the condition
Monoblastic
73
1) Auer rods
2) AML t (15;17)
What's the condition?
Acute promyelocytic leukemia
74
Describe the etiology of Lymphocytic predominance non-classical Hodgkin Lymphoma
Rare, Usually in males under 35yrs
75
Describe the common findings of Lymphocytic predominance non-classical Hodgkin Lymphoma
A mix of lymphocytes & histocyte variants (aka Popcorn cells)
76
Describe the immuno-markers for Lymphocytic predominance non-classical Hodgkin Lymphoma
1) CD20+ & BCL-6 +
2) CD15 - & CD30 -
3) EBV -
77
1) CD20+ & BCL-6 +
2) CD15 - & CD30 -
3) EBV -
Indicate which condition?
Lymphocytic predominance non-classical Hodgkin Lymphoma
78
Describe the etiology of Lymphocyte depletion-type Hodgkin Lymphoma
The rarest, usually older men who are HIV + & from 3rd world countries
79
Describe the common findings of Lymphocyte depletion-type Hodgkin Lymphoma
1) Low lymphocyte #
2) High Reed-Sternberg cell #
3) High association with EBV
4) Poorest prognosis
80
1) Low lymphocyte #
2) High Reed-Sternberg cell #
3) High association with EBV
4) Poorest prognosis
Are indicating of which condition?
Lymphocyte depletion-type Hodgkin Lymphoma
81
Describe the immuno-markers of Lymphocyte depletion-type Hodgkin Lymphoma
1) CD15+ & CD30+
2) EBV +
82
1) CD15+ & CD30+
2) EBV +
Are indicative of which condition?
Lymphocyte depletion-type Hodgkin Lymphoma
83
Describe the etiology of Lymphocyte-rich Hodgkin Lymphoma
Older men
84
Describe the common findings of Lymphocyte-rich Hodgkin Lymphoma
1) Reed-Sternberg cells
2) Mononuclear variants (halo cells)
3) More reactive lymphocytes
85
1) Reed-Sternberg cells
2) Mononuclear variants (halo cells)
3) More reactive lymphocytes
Are common findings of which condition?
Lymphocyte-rich Hodgkin Lymphoma
86
Describe the immuno-markers of Lymphocyte-rich Hodgkin Lymphoma
1) CD15+ & CD30+
2) EBV -
87
1) CD15+ & CD30+
2) EBV -
Are indicative of which condition?
Lymphocyte-rich Hodgkin Lymphoma
88
Describe the etiology of Mixed cellular type Hodgkin Lymphoma
Usually males, it has to peaks of incidence
- Young adulthood
- Over 55yrs old
89
Describe the common findings of Mixed cellular type Hodgkin Lymphoma
A heterogenous mix of cells:
- T cells
- Eosinophils
- Benign macrophages
- Reed-Sternberg cells
- Lacunar cells
- Mononuclear lacunar cells
90
A heterogenous mix of cells:
- T cells
- Eosinophils
- Benign macrophages
- Reed-Sternberg cells
- Lacunar cells
- Mononuclear lacunar cells
Are common findings of which condition?
Mixed cellular type Hodgkin Lymphoma
91
Describe the immunomarkers of Mixed cellular type Hodgkin Lymphoma
1) CD15+ & CD30+
2) EBV+
3) Heterogenous mix of cells
92
1) CD15+ & CD30+
2) EBV+
3) Heterogenous mix of cells
Indicate which condition?
Mixed cellular type Hodgkin Lymphoma
93
Describe the clinical features of Mixed cellular type Hodgkin Lymphoma
1) Painless lymphadenopathy
2) Fever
3) Night sweats
4) Weight loss
94
1) Painless lymphadenopathy
2) Fever
3) Night sweats
4) Weight loss
In a condition with heterogenous mix of cells indicates....
Mixed cellular type Hodgkin Lymphoma
95
Describe the etiology of Nodular sclerotic type Hodgkin Lymphoma
Usually young women
96
Describe the common findings of Nodular sclerotic type Hodgkin Lymphoma
1) Lacunar variant Reed-Sternberg cells
2) Fibrous bands & nodular architecture
3) Good prognosis usually
97
1) Lacunar variant Reed-Sternberg cells
2) Fibrous bands & nodular architecture
3) Good prognosis usually
Are common findings of which condition?
Nodular sclerotic type Hodgkin Lymphoma
98
Describe the immuno-markers of Nodular sclerotic type Hodgkin Lymphoma
1) CD15+ & CD30+
2) EBV -
3) Common in young women
99
1) CD15+ & CD30+
2) EBV -
3) Common in young women
Indicate which condition?
Nodular sclerotic type Hodgkin Lymphoma
100
Describe the clinical features of Nodular sclerotic type Hodgkin Lymphoma
Mediastinal mass & supraclavicular involvement
101
Describe the etiology of Follicular (Bcell) Lymphoma
Usually middle aged men/women
102
Describe the common findings of Follicular (Bcell) Lymphoma
1) t(14;18) translocation on IgH (heavy) & Bcl-2
2) Overexpression of Bcl-2
3) No mantle zone
4) No interfollicular space
5) Centrocytes (small/cleaved cells)
6) Centro blasts (large cells)
103
1) t(14;18) translocation on IgH (heavy) & Bcl-2
2) Overexpression of Bcl-
3) No mantle zone
4) No interfollicular space
5) Centrocytes (small/cleaved cells)
6) Centro blasts (large cells)
Are all common findings of which condition?
Follicular (Bcell) Lymphoma
104
3) No mantle zone
4) No interfollicular space
5) Centrocytes (small/cleaved cells)
6) Centro blasts (large cells)
Indicate which condition?
Follicular (Bcell) Lymphoma
105
Describe the immuno-markers of Follicular (Bcell) Lymphoma
1) CD5- & CD23-
2) Bcl-2-
3) CD19+, CD20+, & CD10+
106
1) CD5- & CD23-
2) Bcl-2-
3) CD19+, CD20+, & CD10+
Indicate which condition?
Follicular (Bcell) Lymphoma
107
Describe the clinical feature of Follicular (Bcell) Lymphoma
Painless "waxing & wanning" lymphadenopathy
108
Describe the etiology of Burkitt's lymphoma
usually children & young adults
109
Describe the causes of Burkitt's lymphoma
All three types are associated with heavy Ig t(8;14) translocations & MYC-C translocations which increase the overall expression of myc proteins
110
All three types are associated with heavy Ig t(8;14) translocations & MYC-C translocations which increase the overall expression of myc proteins
The cause for which condition?
Burkitt's lymphoma
111
Describe the histological findings in Burkitt's lymphoma
Medium sized lymphoma with diffuse infiltrating cells that have round/oval nuclei, coarse chromatin, & several nucleoli
112
What's the condition?
Burkitt's lymphoma
113
Describe the common findings of Burkitt's lymphoma
1) medium sized lymphoma with diffuse infiltrates
2) Tumor has a high mitotic index
3) Tumor has a "starry-sky" appearance (macrophages)
114
1) medium sized lymphoma with diffuse infiltrates
2) Tumor has a high mitotic index
3) Tumor has a "starry-sky" appearance (macrophages)
Are all common findings of which condition?
Burkitt's lymphoma
115
Describe the immuno-markers of Burkitt's lymphoma
1) IgM
2) CD19+ & CD20+
3) CD10+
4) Bcl-6 +
5) Usually Bcl-2 -
116
1) IgM
2) CD19+ & CD20+
3) CD10+
4) Bcl-6 +
5) Usually Bcl-2 -
Indicate which condition?
Burkitt's lymphoma
117
Describe the African/Endemic subtype Burkitt's lymphoma
1) Has a STRONG ass with EBV (+)
2) Presents as a jaw mass
3) Involves abdominal viscera (kidneys, ovaries, & adrenals)
4) Involves t(8;14) of heavy Ig & myc-c
118
1) Has a STRONG ass with EBV (+)
2) Presents as a jaw mass
3) Involves abdominal viscera (kidneys, ovaries, & adrenals)
4) Involves t(8;14) of heavy Ig & myc-c
Indicates which subtype of Burkitt's lymphoma
African/Endemic subtype Burkitt's lymphoma
119
Describe the Western/Sporadic subtype of Burkitt's lymphoma
1) Not ass with EBV (-)
2) Manifests as a mass in the ileocecal valve, right iliac fossa, or peritoneum
3) Biopsy shows a starry sky app
4) Patient usually has Gi distress
120
1) Not ass with EBV (-)
2) Manifests as a mass in the ileocecal valve, right iliac fossa, or peritoneum
3) Biopsy shows a starry sky app
4) Patient usually has Gi distress
Indicates which subtype of Burkitt's lymphoma
Western/Sporadic subtype of Burkitt's lymphoma
121
Describe the Immunodeficiency subtype of Burkitt's lymphoma
presents in the lymph nodes of immunocompromised patients
122
presents in the lymph nodes of immunocompromised patients
Is indicative of which subtype of Burkitt's lymphoma
Immunodeficiency subtype of Burkitt's lymphoma
123
Describe the etiology of Hodgkin lymphoma (Bcell lymphoma)
Typically young adults & adolescents (average age is 32yrs @ diagnosis)
124
Describe the common findings of Hodgkin lymphoma (Bcell lymphoma)
1) Reed-Sternberg cells (present in all classic HL)
2) It can be associated with or without EBV infection (target Reed-Sternberg cells)
125
1) Reed-Sternberg cells (present in all classic HL)
2) It can be associated with or without EBV infection (target Reed-Sternberg cells)
Are common findings of which condition?
Hodgkin lymphoma (Bcell lymphoma)
126
Describe the features of Reed-Sternberg cells
1) Have an OWL's eyes app
2) CD15+ & CD30+
3) CD45-
4) Release IL-5, IL-10, M-CSF, & Eotaxin (chemokine) to increase reactive leukocytes, macrophages & granulocytes in the tumor
5) Use the PD ligand to evade the immune system (by inhibiting CD8-Tcell apoptosis)
127
1) Have an OWL's eyes app
2) CD15+ & CD30+
3) CD45-
4) Release IL-5, IL-10, M-CSF, & Eotaxin (chemokine) to increase reactive leukocytes, macrophages & granulocytes in the tumor
5) Use the PD ligand to evade the immune system (by inhibiting CD8-Tcell apoptosis)
Are all features of which cell
Reed-Sternberg cells
128
Describe the 3 main causes of Hodgkin lymphoma (Bcell lymphoma)
1) EBV targets cells that express CD21 (EBV receptor) & invade them via LMP to infect B-cells and increase NF-kB & proliferation)
2) A mutated REL protooncogene increases NF-kB production (increases Bcell prolif)
3) EBV(-) cells in the LN of patients with Mono can use EBV to turn B cells into Reed-Sternberg cells)
129
1) EBV targets cells that express CD21 (EBV receptor) & invade them via LMP to infect B-cells and increase NF-kB & proliferation)
2) A mutated REL protooncogene increases NF-kB production (increases Bcell prolif)
3) EBV(-) cells in the LN of patients with Mono can use EBV to turn B cells into Reed-Sternberg cells)
Describes the 3 main causes of which condition?
Hodgkin lymphoma (Bcell lymphoma)
130
NF-kB activates _______ lineages & JAK+ STAT activate __________ lineages
NF-kB activates lymphoid while
JAK/STAT activate myeloid
131
Describe the clinical features of Hodgkin lymphoma (Bcell lymphoma)
1) Pruritis
2) Fever
3) Diaphoresis
4) Leukocytosis
It mimics acute infection but it's not!
132
1) Pruritis
2) Fever
3) Diaphoresis
4) Leukocytosis
It mimics acute infection but it's not!
Are all clinical features of which condition?
Hodgkin lymphoma (Bcell lymphoma)
133
Describe the cause of tumor lysis syndrome
triggered 1-5 days after chemotherapy
134
Describe the common findings of tumor lysis syndrome
1) Hypocalcemia
2) Hyperuricemia
3) Hyperkalemia
4) Hyperphosphatemia
135
1) Hypocalcemia
2) Hyperuricemia
3) Hyperkalemia
4) Hyperphosphatemia
Are all common findings of which condition?
Tumor Lysis Syndrome
136
Describe the clinical features of Tumor Lysis Syndrome
1) Hypocalcemia & Hyperphosphatemia lead to:
- Neuromuscular irritability & Tetany
2) Hyperuricemia leads to:
- Renal failure, lactic acidosis, & Dehydration
3) Hyperkalemia leads to:
- Ventricular arrythmias & Sudden death
137
1) Hypocalcemia & Hyperphosphatemia lead to:
- Neuromuscular irritability & Tetany
2) Hyperuricemia leads to:
- Renal failure, lactic acidosis, & Dehydration
3) Hyperkalemia leads to:
- Ventricular arrythmias & Sudden death
Are all clinical features of which condition?
Tumor lysis syndrome
138
Describe the etiology of Adult T-cell Leukemia/Lymphoma (ATL)
50-60 yr olds, there's a higher risk in African Americans
139
Describe the 2 causes of Adult T-cell Leukemia/Lymphoma (ATL)
1) An HTLV-1 infection
2) IV drug abuse
140
Describe the common findings in Adult T-cell Leukemia/Lymphoma (ATL)
1) Production of Tax protein (lets HTLV-1 infiltrate cells & activate NF-kB to up Tcell proliferation)
2) Clover/Flower cells (cells with HTLV-1 inside)
141
1) Production of Tax protein (lets HTLV-1 infiltrate cells & activate NF-kB to up Tcell proliferation)
2) Clover/Flower cells (cells with HTLV-1 inside)
Are common findings of which condition?
Adult T-cell Leukemia/Lymphoma (ATL)
142
Describe the clinical features of Adult T-cell Leukemia/Lymphoma (ATL)
1) Skin lesions
2) Generalized lymphadenopathy
3) Hepatosplenomegaly
4) Peripheral blood lymphocytosis
5) Hypercalcemia
143
1) Skin lesions
2) Generalized lymphadenopathy
3) Hepatosplenomegaly
4) Peripheral blood lymphocytosis
5) Hypercalcemia
Are all clinical features of which of the following condition?
Adult T-cell Leukemia/Lymphoma (ATL)
144
Describe the etiology of Diffuse large Bcell lymphoma
It can happen in children & young adults but it's more common in older persons (~60yrs)
145
Describe the causes of Diffuse large Bcell lymphoma
1) Dysregulation of Bcl-6 (DNA-binding zinc finger transcription repressor) causing unregulated proliferation
2) t(8;14) translocation causing overexpression of Bcl-2 (inhibits apoptosis)
146
1) Dysregulation of Bcl-6 (DNA-binding zinc finger transcription repressor) causing unregulated proliferation
2) t(8;14) translocation causing overexpression of Bcl-2 (inhibits apoptosis)
Causes of which condition?
Diffuse large Bcell lymphoma
147
Describe the clinical features of Diffuse large Bcell lymphoma
1) Presents as a large extra nodal mass
2) Has very large cells with a diffuse growth pattern
148
1) Presents as a large extra nodal mass
2) Has very large cells with a diffuse growth pattern
Indicates which condition?
Diffuse large Bcell lymphoma
149
Describe the etiology of mantle cell lymphoma
Usually older men aged 50-60/+ yrs
150
Describe the common findings in mantle cell lymphoma
t(8;14) translocation of heavy IgH to increase expression of Cyclin D1
151
t(8;14) translocation of heavy IgH to increase expression of Cyclin D1
Findings indicate which condition?
Mantle cell lymphoma
152
Describe the clinical features of Mantle cell lymphoma
1) Painless Generalized lymphadenopathy
2) Spleen or peyer's patch involvement
3) Incurable (3-4yr survival time)
153
1) Painless Generalized lymphadenopathy
2) Spleen or peyer's patch involvement
3) Incurable (3-4yr survival time)
Clinical features indicate which condition?
Mantle cell lymphoma
154
Describe the immunomarkers of Mantle cell lymphoma
1) Very high cyclin D1
2) CD5+
3) CD23-
4) CD19+ & CD20+
155
1) Very high cyclin D1
2) CD5+
3) CD23-
4) CD19+ & CD20+
indicate which condition?
Mantle cell lymphoma
156
Describe the etiology of small cell lymphocytic lymphoma
usually 60/+ yr old men
157
Describe the common findings of small cell lymphocytic lymphoma
1) Elimination of nodal architecture
2) Increase in prolymphocytes
3) Effects lymph nodes
158
1) Elimination of nodal architecture
2) Increase in prolymphocytes
3) Effects lymph nodes
Are common findings of which condition?
small cell lymphocytic lymphoma
159
Describe the clinical features of small cell lymphocytic lymphoma
1) Asymptomatic at diagnosis
2) Enlargement of LN
3) Weight-loss/anorexia
4) Fatigue
5) Generalized lymphadenopathy
6) Hepatosplenomegaly
160
1) Asymptomatic at diagnosis
2) Enlargement of LN
3) Weight-loss/anorexia
4) Fatigue
5) Generalized lymphadenopathy
6) Hepatosplenomegaly
Are all clinical findings of which condition?
small cell lymphocytic lymphoma
161
Describe the etiology of Chronic lymphocytic leukemia
usually older men 60yrs/+
162
Describe the common findings of Chronic lymphocytic leukemia
1) An absolute lymphocyte # >5000 ***
2) Smudge cells
3) Proliferation centers
163
1) An absolute lymphocyte # >5000 ***
2) Smudge cells
3) Proliferation centers
Are common findings of which condition?
Chronic lymphocytic leukemia
164
Describe the causes of Chronic lymphocytic leukemia
Deletions of the following:
- 13q14.3
- 11q1
- 17p
- XXX12q
165
Deletions of the following:
- 13q14.3
- 11q1
- 17p
- XXX12q
Causes indicate which condition?
Chronic lymphocytic leukemia
166
Describe the immunomarker of Chronic lymphocytic leukemia
1) Pan Bcell (CD19+ & CD20+)
2) CD5+ & CD23+
3) CD10-
167
1) Pan Bcell (CD19+ & CD20+)
2) CD5+ & CD23+
3) CD10-
Indicate which condition?
Chronic lymphocytic leukemia
168
In Chronic lymphocytic leukemia which gene deletion is associated with the poorest prognosis?
17p
169
Describe clinical features of Chronic lymphocytic leukemia
1) Asymptomatic at diagnosis
2) Non-specific symptoms (weight-loss, anorexia, & fatigue)
3) Generalized lymphadenopathy
4) Hepatosplenomegaly
170
1) Asymptomatic at diagnosis
2) Non-specific symptoms (weight-loss, anorexia, & fatigue)
3) Generalized lymphadenopathy
4) Hepatosplenomegaly
Are clinical features of which condition?
Chronic lymphocytic leukemia
171
Describe the 3 main complications of Chronic lymphocytic leukemia
1) Warm (IgG) or cold (IgM) Ab-autoimmune hemolytic anemia
2) Hypogammaglobulinemia (high bacterial infection risk)
3) It can progress to aggressive diffuse large B cell lymphoma (Richter syndrome)
172
Describe the etiology of plasma cell myeloma (multiple myeloma)
Older adult men, usually 65-70yrs olds, being African American is a bigger risk
173
Describe the causes of plasma cell myeloma (multiple myeloma)
1) Rearrangements of IgH (x6p21) with cyclin D3
2) Deletions of 17p (p53 gene)
Both lead to unregulated proliferation of neoplastic plasma cells that make too much M protein (IgG or IgA or kappa or lambda light chain variants)
174
1) Rearrangements of IgH (x6p21) with cyclin D3
2) Deletions of 17p (p53 gene)
Both lead to unregulated proliferation of neoplastic plasma cells that make too much M protein (IgG or IgA or kappa or lambda light chain variants)
Causes indicate which condition?
plasma cell myeloma (multiple myeloma)
175
Describe the common findings of plasma cell myeloma (multiple myeloma)
1) High IL-6 (Growth factor for plasma cells)
2) High IL-7 (Osteoclast activator)
3) High monoclonal IgG spike (Kappa) or IgA
4) High levels of Bence-Jones protein (casts in kidneys)
5) Hyperglobulinemia (high serum globins & proteins)
6) High ESR
7) Rouleaux formation of RBCs (poker chip)
176
1) High IL-6 (Growth factor for plasma cells)
2) High IL-7 (Osteoclast activator)
3) High monoclonal IgG spike (Kappa) or IgA
4) High levels of Bence-Jones protein (casts in kidneys)
5) Hyperglobulinemia (high serum globins & proteins)
6) High ESR
7) Rouleaux formation of RBCs (poker chip)
Are all common findings of which condition?
plasma cell myeloma (multiple myeloma)
177
Describe the clinical features of plasma cell myeloma (multiple myeloma)
1) "CRAB"
Calcification
hypeRcalcemia
Anemia
"punched out" Bone lytic lesions (Severe bone pain)
2) Osteopenia (diffuse mineralization)
Tumors in axial skeleton
3) Leukopenia
4) Thrombocytopenia
5) Renal insufficiency with azokmia
6) Primary amyloidosis (Puffy face, confusion, periorbital edema)
178
1) "CRAB"
Calcification
hypeRcalcemia
Anemia
"punched out" Bone lytic lesions (Severe bone pain)
2) Osteopenia (diffuse mineralization)
Tumors in axial skeleton
3) Leukopenia
4) Thrombocytopenia
5) Renal insufficiency with azokmia
6) Primary amyloidosis (Puffy face, confusion, periorbital edema)
Are all clinical findings of which condition?
plasma cell myeloma (multiple myeloma)
179
Describe the immunomarkers of plasma cell myeloma (multiple myeloma)
1) CD138 (syndecan)
2) CD56+
180
1) CD138 (syndecan)
2) CD56+
Are indicative of what condition?
plasma cell myeloma (multiple myeloma)
181
In plasma cell myeloma (multiple myeloma) Rouleaux formations of RBCs are due to high levels of ____
IgG
182
In plasma cell myeloma (multiple myeloma) what indicates a poor prognosis?
High levels of IL-6
183
How is plasma cell myeloma (multiple myeloma) able to persist in the host?
The tumor increases IL-6 levels, which trigger RANKLE to activate osteoclasts (IL7)
The osteoclasts lyse the bone matrix causing an increase in
-MIP-1alpha (tumors on axial skeleton)
-FRP-2 (calcification
- WNT (punched out lesions
184
Which bones are at risk of being affected by plasma cell myeloma (multiple myeloma)
1. Spine
2. Ribs
3. Skull
4. Pelvis
5. Femur
6. Clavicle
7. Scapulae
185
Describe the 3 stages of progression of mycosis fungiodes (cutaneous Tcell lymphoma)
1. Premycotic (erythematous, eczematoid, &/or psoriasiform rashes)
2. Plaques (raised)
3. Tumors
186
1. Premycotic (erythematous, eczematoid, &/or psoriasiform rashes)
2. Plaques (raised)
3. Tumors
the 3 stages of progression of mycosis fungiodes (cutaneous Tcell lymphoma)
187
Describe the common findings of mycosis fungiodes (cutaneous Tcell lymphoma)
1) High CLA (Cutaneous leukocyte antigen)
2) High CCR4 & CCR10 (Chemokines)
3) Neoplastic T cells with cerebriform nucleus
4) Halo cells & spongiosis (Pautier misprocesses
188
1) High CLA (Cutaneous leukocyte antigen)
2) High CCR4 & CCR10 (Chemokines)
3) Neoplastic T cells with cerebriform nucleus
4) Halo cells & spongiosis (Pautier misprocesses
Common findings indicate which condition?
mycosis fungiodes (cutaneous Tcell lymphoma)
189
Describe what Sezary syndrome is
A Cutaneous T cell variant that manifests as a generalized exfoliative erythroderma which can progress to an aggressive T cell lymphoma
190
A Cutaneous T cell variant that manifests as a generalized exfoliative erythroderma which can progress to an aggressive T cell lymphoma
Describes which condition?
Sezary syndrome
191
What are the common findings with Sezary syndrome?
1) High serum IgE
2) Sezary cell
3) Redness
192
Describe the etiology of Langerhans cell histiocytosis (Histiocytosis X)
Usually male children
193
Describe the common findings of Langerhans cell histiocytosis (Histiocytosis X)
1) Dendritic/Langerhans cells in the skin that express S-100 & CD1A
2) Birbeck granules (tennis rackets)
194
1) Dendritic/Langerhans cells in the skin that express S-100 & CD1A
2) Birbeck granules (tennis rackets)
Indicate which condition?
Langerhans cell histiocytosis (Histiocytosis X)
195
Describe the Eosinophilic granule subtype of Langerhans cell histiocytosis (Histiocytosis X)
A unifocal condition that affects the medullary cavities of bones (skull, ribs, & femur)
Patient (young 10-12yrs) experiences severe bone pain
196
A unifocal condition that affects the medullary cavities of bones (skull, ribs, & femur)
Patient (young 10-12yrs) experiences severe bone pain
Describes which subtype of Langerhans cell histiocytosis (Histiocytosis X)?
Eosinophilic granule
197
Describe Hand-Schuller Christian triad subtype of Langerhans cell histiocytosis (Histiocytosis X)
A multifocal condition usually affecting young (6-10yrs) that causes:
-Multiple boney masses
- Diabetes insipidus (post pituitary)
- Exophthalmos (post pituitary)
- Headaches
- Bloody vision
- Lytic bone lesions
198
A multifocal condition usually affecting young (6-10yrs) that causes:
-Multiple boney masses
- Diabetes insipidus (post pituitary)
- Exophthalmos (post pituitary)
- Headaches
- Bloody vision
- Lytic bone lesions
Describes which subtype of Langerhans cell histiocytosis (Histiocytosis X)?
Hand-Schuller Christian triad subtype
199
Describe the Letterer-Siwe disease subtype of Langerhans cell histiocytosis (Histiocytosis X)
A multifocal condition that appears before 2yrs old & causes:
- Cutaneous lesions (Seborrheic eruptions on trunk & scalp)
- Hepatosplenomegaly
- Lymphadenopathy
- Pulmonary lesions
- Destructive osteolytic bone lesions
- Recurrent infections (Otitis media & mastoiditis)
200
A multifocal condition that appears before 2yrs old & causes:
- Cutaneous lesions (Seborrheic eruptions on trunk & scalp)
- Hepatosplenomegaly
- Lymphadenopathy
- Pulmonary lesions
- Destructive osteolytic bone lesions
- Recurrent infections (Otitis media & mastoiditis)
Describes which subtype of Langerhans cell histiocytosis (Histiocytosis X)?
Letterer-Siwe disease subtype
201
Describe the etiology of Chronic Myelogenous leukemia
A broad age range but it peaks in the 45-85yrs gap (64 being the median)
202
Describe the causes of Chronic Myelogenous leukemia
BCR-ABL combination (due to a t(9;22) translocation (Philadelphia)) it activates BCR-ABL tyrosine kinase
203
BCR-ABL combination (due to a t(9;22) translocation (Philadelphia)) it activates BCR-ABL tyrosine kinase
Cause indicates which condition?
Chronic Myelogenous leukemia
204
Describe the common findings of Chronic Myelogenous leukemia
1) Band cells
2) Blast cells
3) LOW LAP
4) Hypercellular bone marrow
5) High eosinophils, basophils, megakaryocytes, & neutrophils
6) Scattered macrophages (Abundant Sea blue histocytes)
7) Leukocytosis of 100,000
205
1) Band cells
2) Blast cells
3) LOW LAP
4) Hypercellular bone marrow
5) High eosinophils, basophils, megakaryocytes, & neutrophils
6) Scattered macrophages (Abundant Sea blue histocytes)
7) Leukocytosis of 100,000
Are all common findings of which condition?
Chronic Myelogenous leukemia
206
Describe the etiology of T cell Acute lymphoblastic Leukemia/Lymphoma
Usually male children & adolescents ages 8-14yrs old
207
Describe the Causes of T cell Acute lymphoblastic Leukemia/Lymphoma
A gain of function mutation in NOTCH1 (needed for Tcell development)
208
A gain of function mutation in NOTCH1 (needed for Tcell development)
Cause is indicative of which condition?
T cell Acute lymphoblastic Leukemia/Lymphoma
209
Describe the features of T cell Acute lymphoblastic Leukemia/Lymphoma
1) Mediastinal thymic masses
2) Lymphadenopathy
3) Splenomegaly
210
1) Mediastinal thymic masses
2) Lymphadenopathy
3) Splenomegaly
Describes which condition?
T cell Acute lymphoblastic Leukemia/Lymphoma
211
Describe the immunomarkers of T cell Acute lymphoblastic Leukemia/Lymphoma
1) TdT +
2) CD1+, CD2+, CD5+, & CD7+
212
1) TdT +
2) CD1+, CD2+, CD5+, & CD7+
Indicate which condition?
T cell Acute lymphoblastic Leukemia/Lymphoma
213
What are the 2 complications of T cell Acute lymphoblastic Leukemia/Lymphoma
1) Superior vena cava syndrome (facial edema in morning & engorged neck neins)
2) Pancoast tumor (bronchial tumor that presses against Rec. laryngeal nerv)
214
Describe the etiology of Hairy cell Leukemia (B-cell)
Usually middle aged men (whites are the highest risk)
215
Describe the causes of Hairy cell Leukemia (B-cell)
A point mutation in Serine/threonine kinase BRAF gene
216
A point mutation in Serine/threonine kinase BRAF gene
Indicates which condition?
Hairy cell Leukemia (B-cell)
217
Describe the common findings of Hairy cell Leukemia (B-cell)
1) Leukemic cells with fine hair-like filamentous projections (cytoskeleton)
2) Dry tap is needed (due to extensive Bone marrow fibrosis)
3) Tear drop RBCs
218
1) Leukemic cells with fine hair-like filamentous projections (cytoskeleton)
2) Dry tap is needed (due to extensive Bone marrow fibrosis)
3) Tear drop RBC
Common findings indicate which condition?
Hairy cell Leukemia (B-cell)
219
Describe the immunomarkers of Hairy cell Leukemia (B-cell)
1) CD11C+
2) CD25+
3) CD103+
4) TRAP+ (tartrate-resistant-acid-phosphate)
5) BRAF V(valine) 600F(Glutamate)
220
1) CD11C+
2) CD25+
3) CD103+
4) TRAP+ (tartrate-resistant-acid-phosphate)
5) BRAF V(valine) 600F(Glutamate)
Indicate which condition?
Hairy cell Leukemia (B-cell)
221
Describe the clinical features of Hairy cell Leukemia (B-cell)
1) MASSIVE HEPATOSPLENOMEGALY
2) Rare lymphadenopathy
3) Pancytopenia
222
1) MASSIVE HEPATOSPLENOMEGALY
2) Rare lymphadenopathy
3) Pancytopenia
Are features of which condition
Hairy cell Leukemia (B-cell)
223
Describe the etiology of Acute lymphoblastic leukemia (ALL)
Most common in children under 15yrs it peaks at 3yrs, & Hispanics & most responsive to therapy
224
Describe the immunomarkers Acute lymphoblastic leukemia (ALL)
1) TdT +
2) CD10+, CD19+, & CD22+
3) PAX5+
225
1) TdT +
2) CD10+, CD19+, & CD22+
3) PAX5+
Indicate which condition
Acute lymphoblastic leukemia (ALL)
226
Describe the common findings of Acute lymphoblastic leukemia (ALL)
1) Increase lymphoblasts
2) Decreased Hematopoiesis
3) Starry-sky appearance
227
1) Increase lymphoblasts
2) Decreased Hematopoiesis
3) Starry-sky appearance
are all common findings of which condition?
Acute lymphoblastic leukemia (ALL)
228
Describe the common features of Acute lymphoblastic leukemia (ALL)
1) Anemia + fatigue
2) Thrombocytopenia
3) Recurrent infections
4) Bone pain
5) Generalized lymphadenopathy
6) Hepatosplenomegaly
7) Testicular enlargement
8) Headaches
9) Vomiting
10) nerve palsies
229
1) Anemia + fatigue
2) Thrombocytopenia
3) Recurrent infections
4) Bone pain
5) Generalized lymphadenopathy
6) Hepatosplenomegaly
7) Testicular enlargement
8) Headaches
9) Vomiting
10) nerve palsies
Are indicative of which condition?
Acute lymphoblastic leukemia (ALL)
230
What are considered good prognosis factors for Acute lymphoblastic leukemia (ALL)
1) Age 2-10yrs
2) low WBC#
3) t(12;21) rearrangement
4) Hyperdiploidy
231
1) Age 2-10yrs
2) low WBC#
3) t(12;21) rearrangement
4) Hyperdiploidy
are all indicators of a ___ prognosis ofAcute lymphoblastic leukemia (ALL)
GOOD
232
What are considered poor prognosis factors for Acute lymphoblastic leukemia (ALL)
1) Younger than 2ys
2) Presents in adolescences or young adulthood
3) Peripheral blood blast count of over 100,000
233
1) Younger than 2ys
2) Presents in adolescences or young adulthood
3) Peripheral blood blast count of over 100,000
are all indicators of a ___ prognosis of Acute lymphoblastic leukemia (ALL)
Poor
234
Bcell neoplasms immunophenotype
CD19/20+
mature TdT -
immature TdT +
235
Tcell neoplasms immunophenotype
CD3/7+
Mature Tdt -
Immature TdT+
236
Myeloperoxidase immunophenotype
CD33/34+
237
Sepsis
Neutropenia #60,000 & Dohle bodies
238
Leukemoid reaction
WBC >50,000
High LAP
no bands
CD13/15+
CD33/34-
