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PRCE > Hematology q-bank > Flashcards

Hematology q-bank Flashcards

1
Q

Kasabach-Merritt clinical and lab features

A

clinical: giant hemangioma and localized intravascular coagulation
laboratory: thrombocytopenia, consumption coagulopathy (low fibrinogen), microangiopathic hemolytic anemia (schistocytes, RBC fragments)

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2
Q

Kasabach-Merritt treatment options (acute & chronic)

A

acute: platelets, pRBC, FFP, aminocaproic acid
surgical excision, steroids, embolization, radiation therapy, vincristine, cyclophosphamide…
Mortality significant!

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3
Q

Von Willebrand factor

- types and difference & *why it matters re: treatment

A

type 1: quantitatively reduced –> only responder to DDAVP
type 2: qualitatively abnormal
(2a- abnormal protein, 2b- hyperactive protein, binds platelets at lower concentrations and thus can worsen bleeding with DDAVP)
type 3: absent VWF –> 2&3 bleeds treat with recombo FVIII/VWF or cryo

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4
Q

Where does Wilms tumour metastasize to?

A

lungs

occasionally liver

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5
Q

Where does osteosarcoma metastasize to?

A

lungs

bones

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6
Q

Where does neuroblastoma metastasize to?

A 
cortical bone
bone marrow
liver
distant lymph nodes
skin
dura
eyes
rarely pulmonary or intracranial
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7
Q

Where does medulloblastoma metastasize to?

A

leptomeningeal

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8
Q

Hereditary spherocytosis

1) method of transmission
2) clinical features
3) lab features
4) treatment

A

1) AD 75%, new mutation 25%
2) asymptomatic to severe hemolytic anemia, most commonly hemolytic disease of newborn (anemia & hyperbili), anemia, gallstones, splenomegaly, *susceptibility to aplastic crises
3) MCV normal, MCH normal to increased
4) splenectomy at 5-6yrs if severe, folic acid, vaccines for encapsulated organisms

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9
Q

Advantages of leukodepletion in transfusions?

A

reduces virus transmission (*CMV)
reduces alloimmunization to HLA antigens
reduces incidence of febrile transfusion reactions

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10
Q 
What are the following hemoglobins made of?
A
A2
F
HbH
Hb Bart
A 
A - a2b2
A2- a2d2
F- a2g2
HbH- b4
Hb Bart- g4
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11
Q

How many copies of alpha globin?

Describe what happens when they get knocked out & test results.

A

4 alpha globin copies on chr 16
1 missing: silent carrier
2 missing: alpha-Thalassemia trait, lower Hgb concentration in cells= normal Hgb electrophoresis, mild anemia (microcytic, hypochromic), occ HbH incl bodies. diagnose with molecular testing
3 missing: hemoglobin H disease, moderate anemia, hgb electrophoresis show HbH
4 missing: hydrops fetalis, Hb Bart

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12
Q

G6PD

  • mode of inheritance & likely ethnicities
  • describe problem
  • describe crisis
  • most common triggers
A
  • X-linked & Mediterranean, Jews, South Asians
  • G6PD works with NADPH to keep glutathione mopping up free oxidants within RBCs, depletion leads to protein & enzyme damage which lead to hemolysis
  • hemolysis, jaundice
  • Abx (TMP-SMX, nitrofurantoin), antimalarials, methylene blue, ASA, naphthalene, DKA, sepsis, hepatitis…
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13
Q

Cyclophosphamide side effect?

A

hemorrhagic cystitis

can also be caused by other meds, viruses, radiation, amyloidosis, polyoma BK virus in immunosuppressed

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14
Q

Risk factors for cerebrovascular disease in sickle cell?

as per UTD

A

prior TIA
low steady state Hgb (high percentage HgbS)
rate of acute chest syndrome
episode of acute chest within last 2 weeks
elevated systolic blood pressure

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15
Q

Diamond Blackfan anemia

  • mode of inheritance
  • age of presentation
  • clinical features
  • lab features
  • treatment
A
  • AD +/- 50%
  • 2-6 months, 90% <1yr
  • short stature, dysmorphisms 50%
  • normochromic, macrocytic, reticulocytopenia, BM normal, high HgbF, high erythrocyte adenine deaminase
  • steroids, transfusions, SCT
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16
Q

Transient Erythroblastopenia of Childhood

  • age range
  • pathophys
  • lab findings
  • natural history
A

most common red cell aplasia in children!

  • 6mo to 3yrs, mean age at dx 26months
  • ?viral, temporary suppression of erythropoiesis, some neutropenia can occur, thrombocytosis as well
  • recover in 1-2 months
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17
Q

How many copies of beta globin?

Describe what happens when they get knocked out & test results.

A

2 beta globin copies

1 missing: beta-thalassemia minor, mild anemia with low MCV and high RBC. Hemoglobin electrophoresis starts normal but by 1 yr HgbA2 +/- HgbF are elevated. No treatment necessary.
2 missing: beta-thal major or Cooley anemia. Transition from HgbF to A cannot occur so by 1 yr have severe microcytic, hypochromic anemia. They have FTT, HSM, CHF, bone marrow expansion for hematopoiesis, need for chronic transfusions and develop iron overload. SCT to fix.

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18
Q

Beckwith-Wiedeman screening for what and how often?

A
  • hypoglycemia, with random and for symptoms during first days of life
  • screen for developmental issues
  • Wilms tumour and hepatoblastoma: abdominal ultrasound q3 months until 8 years with serum AFP q2-3 months during first 4 years
  • nephrocalcinosis, nephrolithiasis and medullary sponge kidney: abdo US q1-2 years with urinary calcium:creatinine ratio
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19
Q

Advantages of irradiated blood?

A

Further inactivates donor cells to reduce GVHD

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20
Q

Indications for washed pRBCs?

A

history of hemolytic transfusion reaction

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21
Q

Good prognostic indicators for ALL?

A

rapid response to therapy

cellular indices like hyperdiploidy, trisomies 4 and 10, t12:21, FAB L1 subtype)

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22
Q

Poor prognostic indicators in ALL?

6

A 
age <1 or >10
philadelphia chromosome t9:22
WBC >50,000 at presentation
Mature B-cell leukemia
T-cell leukemia
Monosomies
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23
Q

Genetic conditions predisposing to childhood leukemia

aim for 5

A 
Ataxia-telangiectasia
C(K)linefelters, Kostmann syndrome
Down syndrome
Diamond-Blackfan anemia
nEurofibromatosis1
Fanconi anemia, Li Fraumeni
Schwachman-Diamond syndrome
SCID
PNH
Wiskott Aldrich
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24
Q

Maximum amount of cow’s milk?
Dose of treatment iron?
Duration of iron treatment?

A

<20 oz daily
4-6mg/kg elemental iron
8 weeks after blood values normalize

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25
Q

Anterior mediastinal mass differential diagnosis?

A 
Teratoma
Thymoma
Thyroid tumour
T-cell leukemia
Terrible lymphoma
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26
Q

Middle mediastinal mass differential diagnosis?

A

Lymphoma

Bronchogenic cyst or tumour

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27
Q

Pathognomonic feature of Hodgkin lymphoma

A

Reed-Sternberg cell

28
Q

Most common age group and presentation of Hodgkin lymphoma?

A

15-19 year old

75% present with mediastinal disease

29
Q

Name the tumour location:
Gaze palsy
Multiple cranial nerve palsies
Upper motor neuron deficits

A

Brainstem tumour

30
Q

Name the tumour location:
Focal disorders like motor weakness, sensory changes, speech disorders, seizures and reflex abnormalities
Early hand preference in infants

A

Supratentorial tumours

31
Q

Name the tumour location:

Neuroendocrine deficits: DI, galactorrhea, precocious puberty, hypothyroidism

A

Suprasellar region tumours and third ventricular region tumours

32
Q

Name the tumour location:
Headache, nausea, vomiting and papilledema
Disorders of equilibrium, gait, coordination

A

Classically associated with infratentorial tumours

33
Q

Name the tumour location:

Blurred vision, diploplia and nystagmus

A

Classically associated with infratentorial tumours

34
Q

Name the tumour location:

failure to thrive, emaciation, euphoria, increased appetite

A

Tumour in the diencephalon, just above the brainstem

35
Q

Name the tumour location:

upward gaze palsy, dissociation of pupillary response to light and accommodation, and failure of ocular convergence

A

Parinaud syndrome

Pineal gland tumour

36
Q

ITP therapy options?

3

A

IVIG - rapid rise within 48hrs, s/e headache and vomiting
Anti-D Therapy - for Rh positive patients, induces mild hemolytic anemia and interferes with platelet destruction, rare life-threatening hemolytic cases have occurred
Prednisone - reeeeally ask yourself if you need to do a bone marrow biopsy to rule out ALL before you start

37
Q

Indications for splenectomy in ITP?

2

A

> 4yr old with >1yr and whose symptoms are not easily controlled (that OR Rituximab for chronic ITP)
Life threatening hemorrhage complicating ITP with failure of medical therapy

38
Q 
Risk of transmission of these diseases in blood transfusions?
HIV 
Hep C
Hep B
Parvo
A

HBV 1/1-1.3 million
HCV 1/5-7 million
HIV 1/ 8-12 million
1/5-20 thousand

39
Q

6 year old with eczema, gingival bleeding and recurrent AOMs, pneumonias

  • what inheritance?
  • susceptibility to which infection?
A

Wiskott Aldrich
eczema, thrombocytopenia, recurrent infections particularly with encapsulated organisms

x-linked

40
Q

What are lethal complications of excessive iron stores?

other effects of excessive iron stores?

A
  • congestive heart failure and lethal arrhythmias

- endocrine dysfunction (hypoT4, hypogonadotropic gonadism, GH deficiency, hypoPTH, DM)

41
Q

What factors are involved in the intrinsic pathway?

A

12, 11, 9, 8
contact factors
PTT

42
Q

What factors are in the extrinsic pathway?

A

7
tissue factors
PT

43
Q

What factors are in the common pathway?

A

10, 5, 2, 1
phospholipids
2=prothrombin
1=fibrinogen

44
Q

VWD test abnormality?

A

Maybe none! Otherwise aPTT prolonged

45
Q

Vitamin K dependent factors?

A

2, 7, 9, 10

46
Q

Which organ systems are most commonly involved in Langerhans cell histiocytosis?

A 
BONE (skull, femur, rib, vertebrae)
SKIN (brown to purplish papules, resembles candida)
liver*
spleen*
oral mucosa
lung
bone marrow*
CNS
*indicates worse prognosis
47
Q

Baby with petechiae and bruising, CBC shows severe isolated thrombocytopenia. Remainder of investigations normal including mum’s CBC.

Treatment?
If that fails?

A
  • rescue therapy with PLA-1 negative platelets, IVIG if persistent

Neonatal alloimmune thrombocytopenic purpura (NATP)

  • can be verified by identifying maternal Ab to paternal platelet antigens
  • IVIG +/- steroids to mum in the second trimester if anticipated
  • plan c/section
48
Q

Neonatal ITP treatment?

A

IVIG +/- steroids

Mum’s and baby’s platelets being attacked, often secondary to SLE or ITP

49
Q

What is the Mentzer index?

A

MCV/RBC
if <13 thalassemia more likely
yes! because thalassemias have high RBC, lots of cells, just smaller and less Hgb

50
Q

Neuroblastoma sites of metastasis?

A

lymph nodes, long bones, skull, bone marrow, liver, skin

RARE to lungs and brain

51
Q

Neuroblastoma presenting symptoms?

A

local disease with mass & metastases
Horner syndrome
Paraneoplastic syndrome: opsoclonus-myoclonus
Catecholamine production: sweating, hypertension
Vasoactive peptide production: secretory diarrhea

52
Q

Tumour lysis syndrome findings and treatment?

A

high uric acid, phosphates and potassium
low calcium
Allopurinol to prevent
If TLS established, rasburicase

53
Q

Favourable prognosis factors in neuroblastoma

4

A

stage 1, 2A/2B, 4S
age <1 year
No Myc-N amplification
hyperdiploid and favourable histology

54
Q

chemo man!

C-ears

A

carboplatin & cysplatin:

nephrotoxic & acoustic nerve damage

55
Q

chemo man!

V-arms and legs

A

vincristine:

peripheral neuropathy

56
Q

chemo man!

B-lungs

A

bleomycin:

pulmonary fibrosis

57
Q

chemo man!

D-heart

A

doxorubin:

dilated cardiomyopathy

58
Q

chemo man!

C-kidneys

A

cisplatin & carboplatin:

nephrotoxic and acoustic nerve damage

59
Q

chemo man!

CY-bladder&urethra

A

cyclophosphamide:

hemorrhagic cystitis

60
Q

chemo man!

56 & M for femur

A

5FU, 6-MP, Methotrexate:

myelosuppression

61
Q

What is the formula to calculate an exchange transfusion in a polycythemic newborn?

A

Volume of exchange (mL) = Blood volume (80mL x wt kg) X (observed - desired Hct)/observed Hct

62
Q

Long-term cyclophosphamide side effect?

A

cyclophosphamide - infertility

63
Q

Long-term Hodgkin lymphoma treatment side-effects?

A 
growth retardation
thyroid dysfunction (radiation)
cardiac toxicity (doxo)
secondary malignancy
scoliosis
impaired growth
restrictive lung disease
64
Q

What is associated with del 11p13 (WT1 and PAX6)

A

WAGR syndrome

Wilms tumour, aniridia, genitourinary anomalies and mental retardation

65
Q

What is associated with a WT1 missense mutation?

A

Denys-Drash syndrome

early onset renal failure with mesangial cell sclerosis, male pseudohermaphrodism

PRCE (8 decks)

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