Hematology & Oncology Flashcards

1
Q

Pathological Blood Films

Target Cells
Tear Drop Poikilocytes
Spherocytes
Basophilic Stippling
Howell - Jolly Bodies
Heinz Bodies
Schistocytes
Pencil Poikilocytes
Burr Cells
Acanthocytes
Hypersegmented Neutrophils

A

Target Cells - IDA, Hyposplenism, Sickle Cell / Thalassaemia

Tear Drop Poikilocytes - Myelofibrosis

Spherocytes - Hereditary Spherocytosis, MAHA, Warm AIHA

Basophilic Stippling - Lead Poisoning (Clover Leaf Morphology) , Thalassemia, Sideroblastic Anaemia , Myelodysplasia

Howell - Jolly Bodies AND Pappenheimer bodies - Hyposplenism

Heinz Bodies - G6PD Deficiency, Alpha Thalassaemia

Schistocytes - DIC, Mechanical Heart Valve , Intravascular Haemolysis

Pencil Poikilocytes - IDA

Burr Cells - Pyruvate Kinase Deficiency

Acanthocytes - Abetaproteinemia

Hypersegmented Neutrophils - Megaloblastic Anaemia

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2
Q

Lead Poisoning Associated with what Symptom?

A

Neuropsychiatric
Abdominal Pain
Peripheral Neuropathy

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3
Q

Intravascular Hemolysis Causes ? (So Schistocytes in Blood Film)

A

G6PD Deficiency
Red Cell Fragmentation - TTP, DIC, HUS
PNH
Cold AIHA

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4
Q

Extravascular Hemolytic Anemia Causes? (No schistocytes in blood film)

A

Warm AIHA
Hereditary Spherocytosis
Hemolytic disease of the newborn
Haemoglobinopathy - Sickle Cell, Thalassemia

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5
Q

Triad of HUS

A

MAHA
Thrombocytopenia
Renal Failure AKI

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6
Q

Which Enzymes affected in Lead Poisoning

A

ferrochelatase and ALA dehydratase function.

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7
Q

Treatment for Lead Poisoning ?

A

Chelating Agents
dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol

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8
Q

Drugs that Trigger Acute Intermittent Porphyria

A

Drugs which may precipitate attack
(Blood Problems Have A Sneaky Behaviour)
* Barbiturates
* Pill- OCP
* Halothane
* Alcohol
* Sulphonamides
* Benzodiazepines

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9
Q

Polycythemia Rubra Vera
Diagnostic Criteria

A

Low ESR and Raised Leukocyte ALP

JAK2 Positive + High Hematocrit (>0.52 Males or >0.48 Females) + High Red Cell Mass (>25%) + Mutation in JAK 2

JAK Negative then A1+A2+A3 + 1 more A OR 2 B

A1 - Raised Red Cell Mass OR Hematocrit
A2 - Absence of Mutation in JAK2
A3 - No secondary causes of Erythrocytosis
A4 - Palpable Splenomegaly
A5 - Presence of an acquired genetic abnormality (excluding BCR-ABL) in the haematopoietic cells

B1 - Thrombocytosis
B2 - Neutrophils elevated
B3 - Radiological Splenomegaly
B4 - Low EPO or Low Erythroid Colonies

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10
Q

Where do you see Chocolate Cyanosis ?

A

Methaemoglobinaemia - normal pO2, low SpO2

CO poisoning - low pO2, low or false normal SpO2

Cyanide poisoning - normal pO2 and SpO2

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11
Q

Causes of Hyposplenism (Holly Jowell Bodies and Siderocytes)

A

splenectomy
sickle-cell
coeliac disease,
dermatitis herpetiformis
Graves’ disease
SLE
amyloid

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12
Q

Ann Arbor Staging

A

I = 1 lymph node group
II = >1 lymph node groups, same side of diaphragm
III = >1 lymph node group on different side of diaphragm
IV = Mets

A = no systemic symptoms other than pruritus

B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)

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13
Q

Lugano Classification
(Double Check)

A

Lugano classification

Stage I - SINGLE lymphatic site (i.e., nodal region, Waldeyer’s ring, thymus, or spleen).

Stage IE - single extralymphatic organ or site (IE)

Stage II - 2 or more LN involvement on SAME side of Diaphragm

Stage IIE -Contiguous extra lymphatic extension from a nodal site

Stage III - 2 or More LN on BOTH SIDES of Diaphragm + MAY involve LOCALISED involvement of Spleen IIIS / Extranodal Organ IIIE / Both IIIE+S

Stage IV - DIFFUSE AND DISSEMINATED Organ Spread

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14
Q

Sickle Cell Anemia Crisis Types

A

Thrombotic (triggered by infection, dehydration etc …)

Acute Chest Syndrome - Most Common Cause of Death after Childhood

Anaemia -
Sequestration (Increased CORRECTED Reticulocytes + Splenomegaly)

Aplastic (Reduced CORRECTED Reticulocyte Count + associated with Parvovirus B19 )

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15
Q

Corrected Reticulocyte Count Formula

A

(Patients Hct/ Normal Hct) * % Reticulocyte

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16
Q

Latex Fruit Allergy Associated with

  1. Which Condition
  2. Which Fruits
A

Myelomeningocele spina bifida

Before a night of PASSION-filled MANGO-tango, I put my STRAWBERRY-flavoured condom on my BANANA, careful to leave out my 2 CHESTNUTS.

Then undressed the KIWI girl, expecting the size of PINEAPPLES but ends up with AVACADO’s

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17
Q

Mucosal / GI bleeding more common in Hemophilia A/B or vWD ?

A

Hemophilia B and vWD

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18
Q

Tumor Markers for

  1. Ovarian Cancer
  2. Teratoma
  3. Melanoma / Schwannoma
  4. SCLC, gastric cancer, neuroblastoma
A

CA 125
AFP
S-100
Bombesin

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19
Q

Superior Vena Cava Syndrome Features

A

Facial Swelling
Dyspnea
Visual Disturbance
Early Morning Headaches
Pulseless Jugular Venous Distension

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20
Q

Superior Vena Cava Syndrome Causes

A

SCLC , Lymphoma’s

Metastatic seminoma, Kaposi’s sarcoma
Breast cancer

Aortic aneurysm
mediastinal fibrosis
Goitre
SVC thrombosis

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21
Q

SVCO Management

A

**ONCOLOGICAL EMERGENCY **

Endovascular Stenting

Radical Chemotherapy or Chemo-radiotherapy > Stenting if Lymphoma / SCLC

Glucocorticoids often given although weak evidence

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22
Q

TACO vs TRALI Presentation and Management

A

TACO - HYPERtension + Pulmonary Edema (IV Furosemide + Stop Transfusion)

TRALI - Non Cardiogenic Pulmonary Edema due to activation of host neutrophils increasing vascular permeability

HYPOtension + Hypoxia
(Oxygen + IV fluids for BP + Supportive Care)

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23
Q

Hereditary Angioedema

  1. Acute Attack Treatment
  2. Screening Tests
  3. Prophylactic
  4. Presentation
A
  1. IV C1 Esterase Inhibitor Concentrate Infusion –>
    FFP
  2. C4 is THE reliable test
    C1 Esterase INH is low in Acute Attacks
  3. Anabolic Steroid -Danazol
  4. Painless / Non Pruritic Swelling –> Painful Macular Rash
    Abdominal Pain from Abdominal Visceral Edema
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24
Q

Antiphospholipid Syndrome Treatment in Pregnancy

A

Low Dose Aspirin once Pregnancy Confirmed on Urine

LMWH once FETAL HEART SEEN –> discontinued at 34 weeks

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25
Q

What do we do to minimize vJCD via blood product ?

A
  1. late 1999 onward, all donations have undergone removal of white cells (leucodepletion) in order to reduce any vCJD infectivity present
  2. from 1999, plasma derivatives have been fractionated from imported plasma rather than being sourced from UK donors.
  3. from 2004 onward, recipients of blood components have been excluded from donating blood

Bovine Spongiform Ecephalopathy (BSE)

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26
Q

Associations of VTE

A

Central Venous Catheters (Femoral > Subclavian)
Puerperium
3rd Generation OCP
Hormone Replacement Therapy (E+P >E)
Antipsychotics - Olanzapine

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27
Q

Investigating for Allergies ?

A

Skin prick: food allergies and pollen.

Contraindicated in anaphylaxis and patients on oral antihistamine

Skin patch: contact dermatitis

RAST (Serum Specific IgE) : all others ie food allergies, pollen, venom etc

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28
Q

Warm AIHA

  1. Causes
  2. IgG or IgM associated
  3. What type of Hypersensitivity ?
  4. What’s Evan’s Syndrome ?
  5. Treatment
A

Neoplasm - CLL, NON HODGKIN LYMPHOMA
Connective Tissue Diseases - SLE
Drugs - Ceftriaxone, Piperacillin
HIV or Viral Vaccines
ASSOCIATED WITH PSC

IgG

Type 2 Hypersensitivity

AIHA + Autoimmune Thrombocytopenia = Evans Syndrome

Transfuse INCOMPATIBLE blood
Corticosteroids
Rituximab
Splenectomy
Hematopoietic Stem Cell Transplant

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29
Q

Cold AIHA

  1. Causes
  2. IgG or IgM associated
  3. What type of Hypersensitivity ?
  4. What’s Evan’s Syndrome ?
  5. Treatment
A
  1. Primary (Cold Agglutinin Disease)
    Secondary -

EBV, CMV, Mycoplasma
Lymphoma, Wladenstrom’s
Drugs - Lenalidomide

IgM

COMPLEMENT AND MAC ?????

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30
Q

Causes of Sideroblastic Anemia

A

MALT

Myelodysplasia
Alcohol
Lead Poisoning
TB drugs

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31
Q

Causes of TTP

A

Post Infection
Pregnancy
SLE
HIV
Drugs: Ciclosporin,
OCP, Penicillin, clopidogrel, Acyclovir

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32
Q

Pentad for TTP

A

Fever
Altered Mental Status
Thrombocytopenia
MAHA
AKI

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33
Q

Urine colour changes to dark in which conditions in Hematology

PAPA mnemonic

A

1) Phenylketonuria
2) Alkaptonuria
3) Paroxysmal nocturnal haemoglobinuria
4) Acute intermittent porphyria

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34
Q

Hereditary Spherocytosis

  1. Inheritance
  2. Investigations
A
  1. Autosomal Dominant from Northern Europe
  2. EMA binding test and the cryohaemolysis test

If Atypical Presentation then Electrophoresis

Elevated MCHC

  1. Supportive + Transfusions
    Splenectomy
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35
Q

EBV associated with which Cancers ?

H Pylori Associated Malignancy ?

Protozoal Associated Cancer

A

Nasopharyngeal Cancer
Hodgkin Lymphoma
Burkitt’s Lymphoma

Gastric MALToma - Antral Cancer

Protozoal = Burkitt

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36
Q

Side effect of Anastrozole
MOA
Side Effect

A

Aromatase Inhibitor
Reduces Peripheral Synthesis of Estrogen

ER+ AND Post Menopausal

DEXA scan prior as osteoporosis

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37
Q

Multiple Myeloma Prognostic Marker

A

B2-microglobulin

38
Q

High Risk TLS patients which prophylactic Drug ?

vs Low Risk Patients ?

MOA of these drugs ?

A

High Risk - Rasburicase (Purine (Nephrotoxic) to allantoin (Non Nephrotoxic) converter via Recombinant Urate Oxidase)

Low Risk - Allopurinol
Prevent Uric Acid to Purine Conversion

NEVER GIVE RASBURICASE AND ALLOPURINOL together

39
Q

 Rx of ITP > Oral Prednisolone
 Rx of TTP > Plasma Exchange
 Rx of Hereditary Spherocytosis > Supportive + Folic Acid
 CLL > FCR
 CML > Imitinab
 Waldenstor Gammaglobulinemia > Rituximab
 Lead poisoning > DMSA

40
Q

When conditions are platelet transfusion contraindicated ?

A

Chronic bone marrow failure

Autoimmune thrombocytopenia

Heparin-induced thrombocytopenia

Thrombotic thrombocytopenic purpura

41
Q

Platelet Transfusion Targets

A

Active Bleeding then <30x10^9

Active Bleed into Critical Sites like Orbit or CNS
<100x10^9

42
Q

Leukemoid Reaction vs CML

A

In Leukemoid reaction

High LAP

toxic granulation (Dohle bodies) in the white cells

‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus

Neutrophils Mature have Segmented Nucleus
Eosinophil = Two Lobed Nucleus
Basophil = Bean Shaped Nucleus

43
Q

SVCO Treatment Immediate ?

A

Immediate

Dexamethasone injections
1) Cord compression in extra nodal Non Hodgkin’s lymphoma
2) SVC obstruction

Endovascular Stenting as definitive

44
Q

CLL when to treat

A

Indications for treatment

progressive marrow failure: the development or worsening of anaemia and/or thrombocytopenia

massive (>10 cm) or progressive lymphadenopathy

massive (>6 cm) or progressive splenomegaly

progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months

systemic symptoms: weight loss > 10% in previous 6 months, fever >38ºC for > 2 weeks, extreme fatigue, night sweats

autoimmune cytopaenias e.g. ITP

45
Q

Irradiated Blood Products (T lymphocyte depleted) when to give ?

A

Irradiated if :

  1. Hodgkin
  2. Drugs like : Bendamustine or Fludarabine
  3. Autologous Stem Cell Transplant - 3 months post or 6 months if whole body irradiation given

Allogenic Stem Cell Transplant - Irradiated blood till
1) 6 months post +
2) off immunosuppression 3) No Clinical Evidence of GvHD

46
Q

CMV Negative Blood Products when to give ?

A

1) Intra-uterine transfusions.

2) Neonates up to 28 days post expected date of delivery.

3) Pregnancy.

47
Q

Drugs that cause Pancytopenia

A

ACS

Antibiotics: Trimethoprim, Chloramphenicol.
AED: Carbamazepine
Anti-rheumatoid: Penicillamine, Gold

Carbimazole
Cytotoxics

Sulfonylureas: Tolbutamide

48
Q

Drugs Causing Drug induced Auto-immune Hemolytic Anemia ?

49
Q

Common Pathogen Associated with Neutropenic Sepsis ?

A

Coagulase-negative
Gram-positive
Staphylococcus epidermidis

Neutropenic sepsis
Bacterial peritonitis
Bacterial endocarditis (withing 2 months of Valve replacement surgery)

50
Q

Staphylococcus epidermidis associated with what pathologies ?

A

Neutropenic sepsis
Bacterial peritonitis from Dialysis
Bacterial endocarditis (withing 2 months of Valve replacement surgery)

51
Q

Most Common Symptom Associated with SVCO

52
Q

Factor 5 Leiden Pathophysiology Explain

A

Antithrombin III –> Inhibit 12,11,9 and 7

Protein C and S –> Inhibit 5 & 8

Factor V Leiden - Gain of Function Mutation –> Activated Protein C RESISTANT –> Doesn’t allow Protein C to inactivate Factor 5 –> Thrombosis

53
Q

Most Common Inherited Thrombophilia ?

A

Factor 5 Leiden

54
Q

How does Heparin work ?

A

Stimulates Antithrombin III Inhibiting Factors 12,11,9 and 7

55
Q

Why is Warfarin initially Procoagulant ?

A

Wafarin is a Vit K antagonist (10,9,7 and 2) but ALSO inhibit Protein C and S initially

So give bridging LMWH
Otherwise Wafarin Induced Skin Necrosis can happen

56
Q

Pathophysiology of How Protein C and S are activated ?

A

Thrombin + Thrombomodulin –> Thrombin Thrombomodulin Complex

TTC activated Protein S via Gamma Carboxylation in Liver –> Protein S then Activate Protein C

57
Q

High LAP vs Low LAP conditions

A

Raised in
myelofibrosis
leukaemoid reactions
polycythaemia rubra vera
infections
steroids, Cushing’s syndrome
pregnancy, oral contraceptive pill

Low in
chronic myeloid leukaemia
pernicious anaemia
paroxysmal nocturnal haemoglobinuria
infectious mononucleosis

58
Q

Chemotherapy induced Nausea and Vomiting Antiemetics
(check though again)

A

Give Metoclopramide if Low Risk Unless _________
2nd Line Domperidone
3rd Line Ondansetron + Dexamethasone

Triptan - (An 1- Agonist)
Cyproheptadine (Di - 2) Antagonist
Ondansetrone - (Tri - 3) Antagonist

59
Q

AML Poor Prognostic Factors ?

A

Poor prognostic features AML(mnemonic) :

Age > 60 years

Myeloblast > 20% blasts after first course of chemo

Location by cytogenetics: deletions of chromosome 5 or 7

60
Q

When to discharge patients post anaphylaxis ?

A

2, 6, 12 hours

1 adrenaline shot, 2 OR Previous Biphasic Reaction, >2 OR If Severe Asthma , Late Night Presentation or Slow Release

61
Q

CLL most common Cells to Proliferate ? B Cells or T cells ?

A

CLL - Smudge B cells causing Hypogammaglobinemia

62
Q

BRCA1/2 on Which Chromosomes ?

Which is more associated with Prostate Cancer ?

A

BRCA 1 - 17 (Ovarian)
BRCA 2 - 13 (Prostate)

BRCA 2

63
Q

Gartner Syndrome ?
1. Mutation Which Gene on Which Chromosome ?

  1. Extra-Colonic Manifestations
A

Mutation of APC on Chromosome 5

Skull Osteoma
Thyroid cancer
Epidermoid cysts

64
Q

Cryoglobulinemia

  1. Types
  2. Symptoms (SKIN)
  3. Investigations
  4. Treatment
A

Type 1 -
Monoclonal (IgG or IgM)
MM or Waldenstrom macroglobulinaemia
a/w Raynauds

Type 2
Polyclonal
Hep C / Sjogren / RA / Lymphoma

Type 3
Polyclonal
Sjogren / RA

Skin
T1 - Acrocyanosis , Livedo Reticularis , Raynaud’s

T2 - NON BLANCHING Macules and Papules (due to antibody/antigen deposition within vessels and recruiting complement causing leukocytoclastic vasculitis

Kidney -
MPGN , Thrombosis

Intra-articular -
Mixed - Arthralgia

Nerves -
Mixed - Mononeuritis Multiplex

  1. Low C4 and RA titers in Mixed Type

High ESR

  1. Hep C Treatment

Suppress B cells with Rituximab –> Plasma Cells –> Abnormal Ig

Plasmapheresis

65
Q

Treatment of ITP ? vs Treatment of TTP ?

A

Repeat Platelet count if >30x10^9 + Safety Net (avoid contact sports if <50x10^9)

If
Injury Prone Profession or Impact on QoL
Frequent Symptoms

1st Line Prednisolone (BONE MARROW ASPIRATE BEFORE IF SUS of Malignancy)

2nd Line IVIG

Tranexamic Acid if Menorrhagia

If No response after 3 months of Steroids then consider splenectomy or Mycophenolate

TTP

Plasma Exchange with FFP in the meantime

If Acute / Idiopathic TTP with Cardiac or Neurological Impairment OR REFRACTORY = Rituximab

66
Q

Referral Criteria for ITP ?

A

Nose Bleed >20 mins
Prolonged Gum Bleeding
Hematuria or Malena
Following Trauma to Head

67
Q

ITP causes in Kids vs Adults ?

A

Kids - Post Live Vaccination (MMR)

Adults - Recent Viral Infection

68
Q

In ITP where do the Antibodies attack ?

A

Glycoprotein IIIb/2a
OR
Ib-V-IX complex.

69
Q

Vitamin B12 Deficiency Treatment

A

1mg IM Hydroxocobalamin 3 times a week for 2 weeks then Once / 3 months

Stop once normalized
Life Long if Pernicious or Celiac

If Neurological Impairment :

1mg Hydroxocobalamin IM on alternate days until no further improvement –> 1mg / 2 months for lifelong (if secondary to Pernicious or Celiac)

70
Q

Gold Standard Investigations for Hematological Malignancies (AML,ALL,CML,CLL)

A

ALL- bone marrow biopsy
AML - bone marrow biopsy (look for myeloblasts)
CLL - flow cytometry/ immunophenotyping
CML - FISH/PCR to look for BCR-ABL gene

71
Q

Translocation (11:22) in ?

A

Ewing Sarcoma

72
Q

Facts about Skin Prick , RAST and Skin Patch Testing

A

Skin Prick Test:
1- Easy to perform
2- Inexpensive
3- Use control (Histamine’+’ and Sterile water ‘-‘)
4- Read after 15 min
5- Useful for food allergy and pollen
6- Use diluted antigen and the pierce the skin
7- +Ve test is formation of wheal

RAST:
1- Check amount of IgE to specific Ag
2- Result from 0 ‘-Ve’—– to—– 6’ +Ve’
3- Useful in food allergy, inhaled pollen, bee/wasp
4- Used if skin prick can not be used

Skin Patch Test:
1- Useful for contact dermatitis
2- need dermatologist
3- Read after (48+48)hours
4- Test allergen and irritant

73
Q

When would you investigate for Li-Fraumeni Syndrome ?

A

*Individual develops sarcoma under 45 years
OR
*First degree relative diagnosed with any cancer below age 45 years and another family member develops malignancy under 45 years or sarcoma at any age

74
Q

Polycythemia Rubra Vera associated with Low or High ESR ?

75
Q

How does Celiac Disease Cause Hyposplenism

A

excessive loss of lymphocytes through the inflamed enteric mucosa, leading to spleen’s reticuloendothelial atrophy

76
Q

Which Blood Group is for Universal Donor for RBC and FPP ?

A

O - is universal donor and AB + is universal recipient for whole blood but vice versa for FFP.

77
Q

What organism to Screen before starting Rituximab ?

A

Hepatitis B

78
Q

Capecitabine is the Oral Form of what drug ?

A

5FU - Pyrimidine Antagonist

79
Q

How is CLL (Monoclonal B cell proliferation) different to MM

A

MM
1. A pleuripotent stem cell proliferates –> multiple abnormal plasma cells
2. Cytogenic progression is more common –> risk of transformation to AML
3. More varied B cells –> more varied cytokine release –> more varied systemic features eg osteoclast-activating cytokines causing bony lytic lesions

CLL
1. A single committed B cell proliferates –> monoclonal B cell proliferation
2. Cytogenic progression is less common but a notable risk = Richter’s transformation –> high grade Lymphoma eg DLBCL
3. Body recognizes abnormal B cells and gathers them in the lymph nodes/spleen –> Lymphadenopathy (LAD). Progression of LAD/splenomegaly is one of the indications for treatment.

80
Q

Multiple Myeloma vs MGUS vs Waldenström
Macroglobulinemia

A

MM
IgG > 30 g/l
>10% plasma cells on bone marrow biopsy
Elevated B2 Microglobulin and Lower Albumin (worse prognosis)
+ CRAB

MGUS
<10% plasma cells on bone marrow biopsy
Normal B2 Microglobulin
No CRAB

Waldenström Macroglobulinemia
IgM K subtype (not IgG)
Cryoglobulinemia Type 1 - Acracyanosis, Raynauds Phenomenon, Cold AIHA (DAT+), Hyperviscosity Syndrome Triad ( Visual, Bleeding and Neurological)
HEPATOSPLENOMEGALY unlike MM
No Bone Pain
BUT has Lymphoplasmaocytic Lymphoma + NEUROPATHY (IgM attacks Myeline Sheath)

> 10% Lymphoplasmocytic Cells on Bone Marrow Biopsy - CD19/CD20/CD22 +

Treatment -
Plasmapharesis
Plasma exchange
IVIG
Chemo - Rituximab based

81
Q

Key facts about MM (just in case to remember)

A

MM–> ALL

Type 2 RTA “Proximal” - Fanconi Syndrome

Not seen on Bone Scan - X ray for Lytic Bony Lesions

Multinucleated Giant Cells on Biopsy on BJ deposition in the Tubules (not toxic to glomerulus)

IgGk –> IgA –> K or Lamda

REDUCED Anion Gap Metabolic Acidosis

Beta 2 Microglobulin –> Higher = Worse Prognosis

Albumin Level = Lower = Worse Prognosis

Serum ALP –> Normal

Histopathology = Monoclonal >10% Clock Face Chromatin , Abundant RER , Prominent Golgi , Perinuclear Halo, Peripheral Nucleus

Immunohistochemistry - CD138 +

82
Q

If its not JAK2 mutation for ET then what else is the mutation ?

A

CALR (calreticulin)
If its not JACK its CARL

83
Q

In Sickle Cell Crisis what is the prophylactic drug we give to reduce Crisis ?

A

Hydroxyurea (increase HbF levels) and analgesia managed by Morphine

84
Q

Benign Neutropenia common in which ethnicity ?

A

Black Africans or Afro-Caribbeans

85
Q

Hairy Cell Leukemia Facts

1) Clinical Features
2) Bone Marrow Aspirate
3) What stain

A

1) pancytopenia
splenomegaly
skin vasculitis in 1/3 patients

2) ‘dry tap’ despite bone marrow hypercellularity

3) Tartrate resistant acid phosphotase (TRAP) stain positive

Management

chemotherapy is first-line: cladribine, pentostatin

immunotherapy is second-line: rituximab, interferon-alpha

86
Q

Poor Prognostic Factor for Hodgkin Lymphoma

A

Male >45
Stage 4
WBC > 15000 or Lymphocytes <600
Hb <10.5
Albumin < 40

87
Q

Poor prognostic factors for CLL

A

Male >70
lymphocyte count > 50

prolymphocytes comprising more than 10% of blood lymphocytes

lymphocyte doubling time < 12 months
raised LDH
CD38 +
TP53 mutation

Deletion of Long Arm of 13q = Good Prognosis

Deletion of Short Arm of 17p = Poor Prognosis

88
Q

Treatment for PNH ?

A

Eculizumab (Inhibit C5 Convertase so no MAC)

If Given Neisseria Meningitides Vaccine

Hematopoietic Stem Cell Transplant if Aplastic Anemia

89
Q

Which Gene Mutations in Which Chromosomes and cause what Pathologies

  1. ALK
  2. C-KIT
  3. EGFR
  4. KRAS
A

ALK ch 2 = adenocarcinoma lung
C-KIT ch 4 = (GIST) and AML
EGFR ch 7 =RCC and NSCLC
KRAS. ch 12. = pancreatic cancers

90
Q

Treatment of Methylglobinemia ?

A

Congenital = Ascorbic Acid

If Severe - MethylBlue but CI in G6PD

91
Q

Translocations and Associations

A

Indolent

  1. Follicular -
    T(14:18)
    BCL2
    Hepatosplenomegaly
  2. Marginal Zone -
    BCL10
    H Pylori
    Salivary and Thyroid Gland involvement

Aggressive

  1. Mantle Zone

T(14:11)
BCL1
Cyclin D

  1. Burkitt’s Lymphoma

T(14:8)
cMYC

  1. DLBC

BCL6
EBV + HIV

92
Q

ALL Good vs Bad Prognostic Factors

A

Good - Bad
L1 - L3
Low WBC - High Wbc
Undiff - B or T differentiation
hyperdiploidy-HYPO diploidy
t1:19/11:21 - T9:22
Female - Male
2-10 - <2 or >10