Chapter 13 - WBC Disorders Flashcards
EBV in IM primarily infects 3 organs.
GIMME THOSE FUCKING ORGANS
- Oropharynx
- Liver
- B cells
EBV in IM spreads to B cells in lymph nodes. The virus attaches to CD.. receptors on B cells.
CD21
3 drugs causing neutrophilic leukocytosis (decreased adhesion)
- Corticosteroids
- Catecholamines
- Lithium
Classic Triad in Acute Leukemia
- Fever (infection due to neutropenia)
- Bleeding (thrombocytopenia)
- Fatigue (anemia)
In which type of leukemia can I see thrombocytosis in 35% of cases, unlike other leukemias?
CML.
Other leukemias having thrombosis:
- PV
- ET
2 situations that I have ectopic production pf EPO and so inappropriate absolute polycythemia
- Renal cell CA (MCC)
2. HCC
Which is the risk factor for developing CML?
Exposure to ionizing radiation
MCC of massive splenomegaly in people >50y
MMM
Which myeloproliferative disorder presents with portal HTN and left-sided pleural effusions?
MMM.
PHTN-> due to massive splenomegaly
Left sided pleural effusions-> due to splenic infarcts
T-ALL presents as … (2)
- Anterior mediastinal mass
- ALL
(in a teenager)
B-ALL commonly metastasizes to .. (2)
- CNS
2. Tests
Which is the MC leukemia in Western countries?
CLL
Which is the MCC of generalized lymphadenopathy in people >65y?
CLL
Which leukemia increases the risk for IHA?
CLL
Leukemias (both acute and chronic) more common in males or females?
Males
Translocation in CML?
t(9;22) : BCR-ABL fusion protein-> increased TK activity.
Phil chromosome: chr. 22 with the mutation
BCR: chr. 22
ABL: chr. 9, proto-oncogene
Headache: common in which leukemia?
ALL (CNS involvement)
Which leukemias can transform into AML?
MDSs, CML
JAK2 gene is on which chromosome and affects which WBC disorders?
9p
PV, ET, MMM
MCC of Budd-Chiari syndrome?
PV
Which leukemia can have PUD as a complication?
PV. Increase in mast cells-> increase in Histamine-> increase in Gastrin release
Exact mutation in PV.
JAK2 V617F mutation in exon 14.
All myeloproliferative disorders present with enlarged spleen. Which one of those has enlarged liver too?
PV.
CML-> 8% of cases
Translocation in CML?
t(9;22) : BCR-ABL fusion protein-> increased TK activity.
Phil chromosome.
BCR: chr. 22
ABL: chr. 9, proto-oncogene
Basophilia. Present in…
MPDs. Characteristic for CML.
Which leukemias can transform into AML?
MDSs, CML
Which MPDs can present with Portal HTN?
- PV
2. MMM
Clinical findings in MMM
- Massive Splenomegaly -> maybe: Portal HTN
2. Splenic infarcts -> maybe: left-sided pleural effusions
How is LAP (leukocyte alkaline phosphatase) in
- PV
- CML
- MMM ?
- PV: increased
- CML: decreased
- MMM: normal to increased
How are platelets in Iron Def Anemia?
Increased
In ET, which is more common: bleeding or thrombosis?
Bleeding
Major rick factors (2) for MDSs?
- Alkylating agents
2. Radiotherapy
DDx: Sideroblasts in BM.
- Sideroblastic Anemia
2. MDSs
3 causes of death in MDSs
- Infection
- Bleeding
- Progression to AML
Which leukemia has the worst prognosis?
AML. (5y-survival rate=20%)
[CLL: 5y-survival rate=75%
CML: 5y-survival rate=90%
ALL: best prognosis of all, those with t(12;21). 75-85% are cured, 95%: complete remission]
Alkylating agents: risk factor for which leukemias?
ALL, AML, CLL
In which chromosomal abnormalities can we see AML?
Down s.
Klinefelter s.
Turner s.
Person with Down s. >5y. Risk for which leukemia?
ALL
Cytogenetic abnormalities in AML (2).
- Mutations on chromosome 8
2. t(8;21)
If a patient with CML progresses to AML, will we see Auer Rods in his WBCs?
NO.
Auer-Rods are present in which types of AML?
M2,M3.
Rarely seen in M1, M4.
t(15;17) is seen in which leukemia?
APL (M3 AML)
This translocation causes abnormal retinoic acid metabolism.
Which leukemia has the worst prognosis?
AML. (5y-survival rate=20%)
[CLL: 5y-survival rate=75%
CML: 5y-survival rate=90%
ALL: best prognosis of all. 75-85% are cured, 95%: complete remission]
Which is the MC type of AML (30-40% of cases)?
M2: AML with maturation
Person with Down s.
AML (M7: Acute megakaryocytic)
Person with Down s. >5y. Risk for which leukemia?
ALL
TdT+ is a marker for …
All types of ALL.
t(9;22) is seen in…
Adult ALL more common. Related to poor prognosis.
Risk factors for ALL. (6)
- Antineoplastic agents
- Hodgkin lymphoma
- Ionizing radiation
- Benzene exposure
- MM
- Down s.
t(12;21) is seen in …
Children’s ALL more often. Favorable prognosis
CD10+ (CALLA), TdT+ : diagnosis
Pre-B-cell ALL
CD10-, TdT+, CD7+ : diagnosis
T-cell ALL
Sites of metastasis of B-cell ALLs
- CNS
2. Testicles
Presentation of T-cell ALL
Usually: Mediastinal (thymic) mass -> called Acute Lymphoblstic Lympoma in a Teenager.
WBC disorder associated with HTLV-1
ATLL
In CLL, naive B cells co-express CD.. and CD..
CD5 and CD20
In ATLL and Mycosis Fungoides, there is neoplastic proliferation of mature CD.. T cells
CD4 (helper cells)
TAX gene is activated in …
ATLL. It inhibits p53 suppressor gene.
A person has HSM, hypercalcemia, lytic bone lesions, and a rash. He is (-) for TdT. What WBC disorder has he?
ATLL. (DDx from MM: he has a rash!)
Where do we see lymphocytes with cerebri-form nuclei in a blood smear?
These cells are called Sezary cells.
We see them in Sezary s.
In Mycosis Fungoides, there is neoplastic proliferation of CD4+ T cells. The aggregates of these neoplastic cells are called Pautrier microabscesses. Which is the site of these microabscesses?
The epidermis.
They can form rash, plaques or nodules.
Which is the MC leukemia overall?
CLL
Where do I see smudge cells?
CLL
Where do I see CD5+,CD20+ B cells?
CLL
What is Richter transformation?
CLL cells can pick up additional mutations and CLL transforms into DLBCL. This is noted clinically by an enlarging lymph node or spleen.
Which WBC disorder can result in Autoimmune vasculitis and arthritis?
HCL
In which WBC disorder lymphadenopathy in uncommon?
HCL
SLL may arise from …
CLL
