Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Rapid Review Step 1 > Hematology & Oncology > Flashcards

Hematology & Oncology Flashcards

1
Q

PNH
(Proximal Nocturnal Hemoglobinuria)

A

Triad of hemolytic anemia, hypercoagulability, and pancytopenia
acquired mutation in the PIGA gene
associated deficiency of CD55 and CD59 complement inhibitor proteins

PNH results in impaired synthesis of Glycosylphospatidylinositol (GPI), resulting in inability to anchor DAF (CD55) & CD59 to the cell membrane and loss of inhibition of complement-mediated lysis of erythrocytes, leukocytes & platelets.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Li-Fraumeni syndrome

A
  • TP53 Mutation
  • Autosomal dominant
  • Caused by inactivating mutation in corresponding tumor suppressor gene
  • Deletion of remaining normal allele (second hit) leads to loss of heterozygosity & malignant transformation
  • Sarcomas, Breast cancer, Brain tumors, Adrenocortical carcinoma, Leukemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Hereditary Spherocytosis

A

RBC membrane defect (Spectrin & Ankyrin), Spherocytes (Increase Osmotic fragility)
1- Hemolytic anemia (dec Hb, Inc LDH)
2- Jaundice
3- Splenomegaly (inc macrophages & congestion)
4- Negative Coombs test
Inc risk of pigmented gallstones & inc risk of aplastic crisis with Parvo virus B-19 infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Henoch-Schönlein
purpura

A
  • IgA vasculitis (affects skin and kidneys)
  • Palpable purpura on buttocks/legs,
  • joint pain,
  • abdominal pain,
  • hematuria
  • Seen in children
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Acute lymphoblastic
leukemia/lymphoma (ALL)

A
  • Age < 15 yrs
  • Gene mutation on Chromosome21
  • Associated with Down syndrome
  • t(12;21) Ž better prognosis;
  • t(9;22) (Philadelphia chromosome) Žworse prognosis.
  • PBS & Bone marrow have↑ Lymphoblasts
  • TdT+, CALLA+
  • CD10+
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Chronic lymphocytic
leukemia (CLL)

A
  • Age > 60 years.
  • Most common adult leukemia. CD20+, CD23+, CD5+ B-cell neoplasm.
  • CLL = Crushed Little Lymphocytes (smudge cells).
    ( Crushed/ Crumpled tissue paper like appearence on histology )
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Hairy cell leukemia

A
  • Adult males.
  • Mature B-cell tumor.
  • Cells have filamentous, hairlike projections (fuzzy appearing on LM)
  • Marrow fibrosis has dry tap on aspiration.
  • Pancytopaneia, Massive Splenomegaly
  • TRAP+, Assosiated w BRAF mutation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Acute myelogenous
leukemia (AML)

A
  • Auer Rods
  • Myeloperoxidase ⊕
  • APL: t(15;17),
  • Responds to all-trans retinoic acid (vitamin A)
  • DIC is a common presentation
  • Associated with Down syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Chronic myelogenous
leukemia (CML)

A
  • Philadelphia chromosome
  • (t[9;22], BCR-ABL)
  • Myeloid stem cell proliferation
  • May transform to AML or ALL (“blast crisis”)
  • Responds to BCR-ABL tyrosine kinase inhibitors (eg, imatinib).
  • Low Leukocytes ALP.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Polycythemia Vera

A
  • Increase in all blood cells
  • ↑ RBC, ↑ WBC, ↑ Platelets
  • JAK2 mutation
  • Intense itching after shower (aquagenic pruritus)
  • ↓ EPO
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Essential
Thrombocythemia

A
  • Massive proliferation of megakaryocytes and platelets.
  • Bleeding and thrombosis
  • ↑ Platelets.
  • 1/3 to 1/2 of essential thrombocythemia patients also have JAK-2 mutation.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Myelofibrosis

A
  • Atypical megakaryocyte hyperplasia
  • Pancytopenia
  • Bone marrow fibrosis (Dry Tap)
  • Massive splenomegaly
  • “Teardrop” RBCs
  • ↓ RBCs, Variable WBCs & Platelets.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Langerhans cell
histiocytosis

A
  • Presents in a child as lytic bone lesions & skin rash or as recurrent otitis media with a mass involving the mastoid bone
  • Express S-100 and CD1a.
  • Birbeck granules
    (“tennis rackets” or rod shaped on EM)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

DIC
Dissemninated Intravascular Coagulaopathy

A
  • Widespread clotting factor activation
  • blood oozing from puncture sites
  • Bleeding complications due to consumption of palelets and clotting factors.
  • Thrombocytopenia (low platelets)
  • ↑ aPTT , ↑ PT , ↑ INR , ↑ D-Dimers
  • Decrease Fibrinogen conc
  • Can be caused by Trauma, Malignancy, Pancreatitis, Toxins, mutliple other causes.

-­↑ bleeding time, ­ ↑ PT, ↑­ aPTT.

  • ↓ Platelet count, ↓ serum fibrinogen, ­ ↑ serum plasmin activity, ­ ↑ serum D-dimer
  • Blood oozing from puncture site= DIC on USMLE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

HUS (Hemolytic-uremic syndrome)

A

In children, Predominately caused by Shiga toxin–producing Escherichia coli (STEC) infection (serotype O157:H7)

  • Thrombocytopenia
  • Hemolytic Anemia
  • Acute kidney Injury
  • Bloody Diarrhea
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

TTP (Thrombotic thrombocytopenic purpura)

A

In Females,
Inhibition or deficiency of ADAMTS13, Inc platelet adhesion and aggregation (microthrombi formation)

“ T A A N “

T = Thrombocytopenia
A = Anemia (Hemolytic)
A = Acute kidney Injury
N = Neurological Symptoms

17
Q

Immune thrombocytopenia

A
  • Destruction of platelets in spleen.
  • Anti-GpIIb/IIIa antibodies –> splenic macrophages phagocytose platelets
  • Inc megakaryocytes on bone marrow biopsy,
  • Decrease platelet count.
  • ↑ Bleeding time, Ⓝ aPTT, Ⓝ PT.
  • platelets are being consumed so ↑ BT & low platelets but coagulation is normal so Ⓝ aPTT, Ⓝ PT.
  • Classical case Child after viral infection or women in 30-40s with random bleeding problem
18
Q

Bernard-Soulier
syndrome

A
  • Defect in adhesion of platelets
  • decr GpIb –> decr platelet-to-vWF adhesion
  • Big platelets
19
Q

Glanzmann
thrombasthenia

A
  • defect in aggregation.
  • decrease GpIIb/IIIa.
  • defective platelet plug formation
20
Q

Iron Def Anemia Vs Anemia of Chronic Dx

A

Anemia of Chronic disease differ from iron deficiency anemia as Iron def has low Ferritin cause the body stores are low, where as in anemia of chronic disease Ferritin levels are high as the body stores are high but iron is bound inside the bone marrow by Hepcidin therefore serum Iron and TIBC are low

21
Q

Lead poisoning

A

Lead inhibits ferrochelatase and ALA dehydratase

     Pneumonic   "LEAD”
  • Lead Lines on gingivae (Burton lines) and on metaphyses of long bones.
  • Encephalopathy and Erythrocyte basophilic stippling.
  • Abdominal colic and sideroblastic Anemia.
  • Drops—wrist and foot drop
22
Q

Wiskott-Aldrich
syndrome

A

Mutation in WAS gene

Pneumonic “WATER”

  • Wiskott-Aldrich:
  • Thrombocytopenia,
  • Eczema,
  • Recurrent (pyogenic) infections

Increase IgE, IgA

23
Q

Acute Intermittent Porphyria

A

Porphobilinogen deaminase def
inc Porphobilinogen
5 P’s
Painful Abd, Port-wine colour urine, Polyneuropathy, Psychological disturbance, Precipitated by drugs.

Management of AIP attack include infusion of Hemin which downregulates hepatic ALA-synthase (Rate limitting enzyme in heme synthesis)

CYP450 inducers (Barbiturates, Antiepileptics, EtOH & Smoking) increase activity of hepatic ALA-synthase

24
Q

Porphyria Cutanea Tarda

A
  • Uroporphyrinogen decarboxylase deficiency,
  • Inc Uroporphyrinogen 3
  • Photosensitivity, Hyperpigmentation, Blisters
  • Exacerbated by Alcohol
  • Causes: Familial & Hep-C
25
Q

Multiple Myeloma

A
  • Most common primary bone tumor in elderly
  • Over productio of IgG (55% cases) > IgA.
  • M-spike with IgG on electrophoresis.
    Pneumonic  “ C R A B “

C = hyperCalcemia
R = Renal Involvement , Rouleaux formation
A = Anemia
B = Bone lesions/ Back pain (lytic lesions, punched out lesions on x-ray)

  • Rouleaux formation RBC stacked like poker chips
  • ↑ ESR
  • Urinanalysis show Ig light chains
    ( Bence-jones protienuira ), —ive urine dipstick

Complications:
-↑ risk of infections,
- 1° Amyloidosis
- Multiple Myeloma is tumor of plasma cells.

26
Q

Waldenstorm Macroglobulenemia

A
  • Overproduction of IgM
  • M-spike with IgM
  • Peripheral Neuropathy
  • No CRAB findings

HYPER VISCOSITY SYNDROME:
- Headache
- Blurry vision
- Raynaud phenomenon
- Retinal hemorrhages

Complications: Thrombosis

27
Q

Myelodysplastic Syndrome

A
  • Seen in older adults.
  • Stem cell disorder –> ineffective hematopoesis
  • Defect in maturation of non-lymphoid lineage
  • Bone marrow blast <20% ( Vs AML blast> 20%)
  • Risk factors: radiation, chemo, benzene
  • Risk of transformation into AML
  • Neutrophils with Bilobed Nucleus.
28
Q

Tumor Lysis Syndrome

A
  • Seen in leukemias/lymphomas, usually caused by treatment initiation
  • Muscle weakness
  • Arythmias (incrse K+)
  • Siezures (decr Ca+2)
  • Acute Kidney Injury (incrse PO4– , incr Uric acid)
29
Q

Hemophilia

A
  • X-Linked Recessive
  • Hemophilia A= Factor VIII deficiency (more common).
  • Hemophilia B= Factor IX deficiency.
  • Ⓝ bleeding time, Ⓝ PT, ­↑ aPTT
  • aPTT is elevated because factors VIII and IX are only in the intrinsic pathway.
  • PT and bleeding time are always normal in hemophilia.
  • classical case school age child with Hemarthrosis (Bleeding into joints).

Rx:
IV desmopressin (DDAVP) can be used as Tx for hemophilia A only (incr production of factor VIII)

30
Q

Von Willebrand Disease (vWD)

A
  • Autosomal dominant condition.
    -↑ Bleeding time, Ⓝ PT, Ⓝ platelet count.
  • Vignettes will almost always give a presentation that entails a mix of one clotting factor problem (i.e., heavy menses, excessive bleeding after tooth extraction) and one platelet problem (i.e., usually mild, cutaneous findings such as petechiae, or epistaxis)
  • vWF has a secondary role where it helps stabilize factor VIII in plasma. Therefore, we sometimes get elevated aPTT in the condition, but that is not necessary aPTT maybe normal or elevated.
  • IV desmopressin (DDAVP) can be used as Tx.
31
Q

Ataxia-Telangectasia

A

Defect in ATM DNA repair gene.

Presents as:
1- Ataxia.
2- Angiomas.
3- IgA deficiency.

32
Q

Lab values in anemia

A
33
Q

Heme Synthesis

A
34
Q

Leukomoid Reaction

A
  • ↑WBCs, ↑Neutrophills, ↑Bands (left shift), (immature neutrophils)
  • ↑Leukocyte ALP
  • Leukomoid reaction can look like Leukemias and have the same labs too except in Leukemias there is low Leukocyte ALP.
35
Q

Hodgkins Lymphoma

A
  • Reed-Sternberg cells (Owl eye nucleus).
  • Localized (single LN).
  • Contiguous.
  • Markers CD30+ & CD15+ are for Reed-sternberg cells & thus hodgkins lymphoma.

B-Symptoms:

  • Low grade fever
  • Night sweats
  • Wt.loss

50% of hodgkins lymphomas are associated with EBV infection, Seen in bimodal age distribution as well as all subtypes are more common in men except Nodular sclerosing type which is 50-50 in men and women & also the most common subtype.

36
Q

Non-Hodgkins Lymphoma

A
  • No Reed-Sternberg cells (Owl eye nucleus)
  • Multiple Nodes are involved
  • Non-contiguous
  • Often associated with HIV infections & Autoimmune diseases.
  • Peak incidence in young adulthood (20-40yrs)
  • Poor prognosis then Hodgkins disease.
  • Types are:
    > B-cells Lymphomas
    > T-cells Lymphomas
37
Q

Burkitts Lymphoma

A
  • B-cell non hodgkins lymphoma.
  • “ Stary Sky “ appearence on histology.
  • C-myc gene translocation, t (8:14), t (8:22), t (2:8)
  • Associated with EBV infection.
  • African child with jaw lesion, or in american seen in adults involving abdomen & pelvis.
38
Q

Tumor Lysis Syndrome

A
  • Seen in leukemias/lymphomas, usually caused by treatment initiation
  • Muscle weakness
  • Arrythmias (incrse K+)
  • Seizures (decr Ca+2)
  • Acute Kidney Injury (incrse PO4– , incr Uric acid)
39
Q

Aplastic Anemia

A
  • Multipotent hematopoietic stem cells are destroyed by cytotoxic Tcells or direct cytotoxic injury→ bone marrow aplasia/hypoplasia→ lack of circulating peripheral blood cells
  • Pancytopenia (Low RBC, WBC, Platelets)
  • Dry Tap on Bone marrow aspiration
  • No Extramedullary hematopoiesis
  • No Splenomegaly
  • Bone marrow biopsy: hypocellular marrow with abundance of stromal & fat cells
  • Causes/ Triggers: Autoimmune, Drugs: cytotoxic chemotherapy, immunosuppressants, idiosyncratic reactions, Ionizing radiation & toxins, Viral infections (eg,viral hepatitis, HIV)

Rapid Review Step 1 (24 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions