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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.

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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

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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

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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

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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+
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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 )
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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
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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
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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.
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10
Q

Polycythemia Vera

A
  • Increase in all
  • ↑ RBC, ↑ WBC, ↑ Platelets
  • JAK2 mutation
  • Intense itching after shower (aquagenic pruritus)
  • ↓ EPO
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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.
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12
Q

Myelofibrosis

A
  • Atypical megakaryocyte hyperplasia
  • Bone marrow fibrosis (Dry Tap)
  • Massive splenomegaly
  • “Teardrop” RBCs
  • ↓ RBCs, Variable WBCs & Platelets.
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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)
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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

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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

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

22
Q

Wiskott-Aldrich
syndrome

A

Mutation in WAS gene
“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.
“ C R A B “
C = hyperCalcemia
R = Renal Involvement , Reaulux 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).

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)

Rapid Review Step 1 (24 decks)

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