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Phase 3a > Haematology > Flashcards

Haematology Flashcards

1
Q

WHEN IN SICKLE CELL DISEASE WOULD YOU USE AN EXCHANGE TRANSFUSION

A

ACUTE CHEST SYNDROME

STROKE

PRIAPISM

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2
Q

WHAT ARE NEONATAL UNCONJUGATED JAUNDICE CAUSES AFTER 2 W

A

BREAST MILK

HYPOTHYROIDISM

GI OBSTRUCTION IE PYLORIC STENOSIS

HAEMOLYTIC ANAEMIA

UTI/INFECTIONS

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3
Q

WHAT IS ABO?

A

WHEN MUM IS BLOOD GROUP O

BABY IS BLOOD GROUP A OR B

MATERNAL ANTIBODIES ATTACK BABYS Hb (ANTI A /ANTI B)

CAUSING HAEMOLYSIS

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4
Q

A PATIENT HAS A Hb COMPOSITION OF HbS AND HbC (although mostly abnormal) WHAT IS THEIR TYPE OF SICKLE CELL DISEASE

A

HbSC

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5
Q

WHAT IS THE PATHOPHYSIOLOGY OF RBC APLASIA

A

DECREASED RETICULOCYTES SO DECREASED LEVELS OF HB AND BILLIRUBIN

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6
Q

WHAT ARE THE TREATEMENTS FOR FANCONI ANAEMIA

A

ANDROGENS

HAEMATIPETIC FACTORS

HAEMATOPOETIC STEM CELL TRANSPLANTS

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7
Q

WHAT IS ALPHA THAMASSEMIA

A

A REDUCTION IN FUNCTIONING ALPA THAMASSEMIA

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8
Q

WHAT IS THE MANAGEMENT OF ALPHA THAMASSEMIA

A

TREATED IN MAJOR:

MONTHLY BLOOD TRANSFUSTIONS

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9
Q

HOW DO YOU DIAGNOSE IMMUNE THROMBOCYTOPENIA PURPURA

A

BLOODS: LOW PLATETLETS,

EXCLUDE:

HIV, CULTURES FOR MENINGITIS, ESR+CRP (SLE), ?BONE MARROW ASPIRATE TO EXCLUDE LEUKEMIA OR BLOOD FILM TO LOOK FOR BLASTOCYSTS

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10
Q

WHAT IS THE PRESENTATION OF FANCONI ANAEMIA

A

INFERTILITY (DECREASED SPERMATOGENESIS)

INCREASED PREDISPOSITION OF INFECTION

ANAEMIA SYMPTOMS

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11
Q

WHAT IS THE PRESENTATION OF BETA THAMASSEMIA

A

PROLONGED NEONATAL JAUNDICE

SEVERE ANAEMIC

FAILURE TO TRIVE

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12
Q

WHAT IS BETA THAMASSEMIA

A

SEVERE REDUCTION IN THE BETA GLOBIN GENE

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13
Q

WHAT IS THE ONGOING MANAGEMENT FOR HAEMOPHILLIA PATIENTS

A

SEVERE: PROFYLACTIC IV FACTORS 8/9

MODERATE: DESMOPRESSIN

MILD: NOTHING

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14
Q

WHAT IS THE PURPOUS OF VON WILLIBRAND FACTOR

A

FACILITATES PLATELET ADHESION TO ENDOTHELIUM WHEN DAMAGED ACTING AS A CARRIER FOR CLOTTING FACTORS (8)SO THAT THEY ARENT INACTIVATED AND THEN CLEARED

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15
Q

DESCRIBE WHAT CAN TRIGGER AN APLASITC CRISIS

A

PARVOVIRUS INFECTION CAUSING DECREASE IN RBC PRODUCTION

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16
Q

WHAT IS THE MANAGEMENT OF SICKLE CELL DISEASE

A

PROPHYLAXIS: FULL IMMS, DAILY PENICILLIN

DAILY FOLIC ACID

AVOIDING TRIGGERS (COLD, DEHYDRATION)

REGULAR OPHALMOGY CHECKS FOR RETINOPATHY

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17
Q

WHAT IS THE PRESENTATION OF SICKLE CELL DISEASE

A

ANAEMIA

JAUNDICE

VASOOCCULUSIVE CRISES’

ACUTE ANAEMIC CRISES’

SPLENOMEGALY

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18
Q

WHEN IS RHESUS INCOMPATIBILITY DIAGNOSED

A

ANTENATALLY

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19
Q

IN PATIENTS WHO HAVE FROQUENCT SICKLE CELL DISEASE CRISES WHAT IS THEIR MANAGEMENT

A

SAME AS NORMAL PLUS

HYDROUREA (INCREASES HbF)

BONE MARROW TRANSPLANT

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20
Q

WHAT IS THE Hb RANGE FOR NEONATES

(ANAEMIA)

A

UNDER 14

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21
Q

WHAT ARE CAUSES OF MACROCYTIC ANAEMIA

A

ALCOHOL (NORMOBLASTIC)

FOLATE (MEGALOBLASTIC)

B12 (PERNACIOUS, MALABSORBTION AND TERMINAL ILEUM)

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22
Q

WHAT IS THE PRESENTATION OF HAEMOPHILLIA

A

PROLONGED BLEEDING

BRUISING

SECURRENT SPONTANEOUS BLEEDS INTO JOINTS AND MUSCLES CAUSING PAIN

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23
Q

WHAT ARE SIGNS OF FANCONI ANAEMIA

A

MICROCYTIC

PANCYTOPENIA

THROMBOCYTOPENIA

NEUTROPENIA

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24
Q

WHAT ARE CAUSES OF MICROCYTIC ANAEMIA

A

IRON DEFICIENCY

THALASSEMIA

SICKLE CELL

CHRONIC DISEASE

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25
Q

WHAT ARE CAUSES OF NORMOCYTIC ANAEMIA

A

PREGNANCY

HAEMOLYTIC ANAEMIAS

APLASTIC

BLOOD LOSS

CHRONIC DISEASE

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26
Q

WHAT IS VON WILLIBRAND DISEASE

A

AN AUTOSOMAL DOMINANT DISEASE CAUSING DEFICIENCY OF VON WILLIBRAND FACTOR

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27
Q

WHAT ARE THE TWO TYPES OF HAEMOPHILLIA

A

ALPHA (FACTOR 8 DEFICIENCY)

BETA (FACTOR 9 DEFICIENCY)

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28
Q

WHAT IS THE TREATEMENT FOR KERNICTUS

A

PHOTOTHERAPY

BLOOD EXCHANGE TRANSFUSIONS

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29
Q

A PATIENT WITH A PMH OF SICKLE CELL DISEASE PRESENTS TO A + E WITH

  • SOB
  • FEVER
  • COUGH
  • PAIN IN CHEST
  • LOW O2 SATS

WHATS IS HER DIAGNOSIS

A

ACUTE CRISIS OF SICKLE CELL DISEASE

  • ACUTE CHEST SYNDROME
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30
Q

WHAT IS THE PRESENTATION OF MAJOR ALPHA THAMASSEMIA

A

OEDEMATUS AND ASCITIS BABY ON 3RD TRIMESTER US

FATAL SHORTLY AFTER BIRTH IN MOST CASES

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31
Q

WHAT SIMPTOM OF HAEMOPHILLIA WOULD CLASSIFY THE HAEMOPHILLIA AS SEVERE

A

SPONTANEOUS BLEEDS INTO JOITS AND MUSCLES

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32
Q

WHAT SHOULD YOU INVESTIGATE FOR IF IRON DEFICIENCY ANAEMIA IS UNRESPONSIVE TO TREATEMENT

A

INVESTIGATE FOR COELIAC, MECKELS DIVERTICULUM, GI BLEEDS,OBG CAUSES

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33
Q

WHAT CAN GIVING IV CLOTTING FACTORS CAUSE AS A COMPLIATION

A

INFECTIONS

DAMAGED PERIFERAL ACCESS

DEVELOPING ANTIBODIES TO FACTORS 8/9

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34
Q

WHAT IS THE MANAGEMENT OF BETA THAMASSEMIA

A

MAJOR AND INTERMEDIA:

MONTHLY BLOOD TRANSFUSIONS

DESFERROXAMINE

BONE MARROW TRANSPLANT

TRAIT:

TREAT AS ABOVE BUT ONLY IF FERRATIN IS LOW

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35
Q

WHEN IS NEONATAL JAUNDICE PHYSIOLOGICAL

A

24 H - 2 W

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36
Q

WHAT IS CRAIG-NAJJAR

A

THERE IS NO GLUCURONILL TRANSFERASE CAUSING ANINCREASE IN UNCONGUGATED BILLIRUBIN

37
Q

WHAT IS THE PRESENTATION OF IMMUNE THROMBOCYTOPENIA PURPURA

A

RED/PURPULE OURPURIC RASH AKIN TO THAT OF MENINGITIS

INCREASED TENDANCY TO BLEED

PETICHEAE

38
Q

WHAT WOULD OCCUR IF BETA THAMASSEMIA WAS LEFT UNTREATED

A

EXTRAMEDULLARY HAEMOPOESIS (MARROW OVERGROWTH)

CAUSING

  • MAXILLARY OVERGROWTH
  • SKULL BOSSING
  • HEPATOSPLENOMEGALY
39
Q

WHAT INVESTIGAIONS ARE REQUIRED IN FANCONI ANAEMIA

A

FBC

MARROW ASPIRATE

GENETIC TESTING

40
Q

HOW IS SICKLE CELL DISEASE SCREENED FOR

A

GUTHRIES TEST (HEEL PRICK)

IN HIGH RISK GROUPS CHORIONIC VILLUS SAMPLING

41
Q

WHAT IS THE MANAGEMENT OF IMMUNE THROMBOCYTOPENIA PURPURA

A

STEROIDS (IV/PO)

AZOTHIOPRINE (IMMUNOSUPPRESSANTS)

ROMIPLOSTIM (THROMBOPOETIN RECEPTOR AGONIST TO STIMULATE PLATELET PRODUCTION)

PLATELET TRANSFUSIONS

SPLENECTOMY

42
Q

HOW DO YOU TREAT RBC APLASIA

A

BLOOD TRANSFUSIONS

STEROIDS

SPLENECTOMY

43
Q

WHAT IS HAEMOLYTIC DISEASE OF THE NEW BORN

A

HBF BEAKS DOWN FASTER THAN HBA CAN REPLACE SO BILLIRUBIN INCREASES

44
Q

WHAT ARE COMPLICATIONS OF THE SPONTANEOUS BLEEDS INTO JOINTS AND MUSCLES

A

ARTHERITIS

45
Q

WHAT INVESTIGATIONS ARE REQUIRED TO DIAGNOSE HAEMOPHILLIA

A

CLOTTING SCREEN LOOKING ESP AT FACTORS 8 + 9

46
Q

WHAT CAN CAUSE A SEQUESTRATION CRISIS IN SICKLE CELL DISEASE

A

ACCUMULATION OF RBC IN SPLEEN

47
Q

WHAT ARE THE THREE TYPES OF ACUTE ANAEMIC CRISIS IN SICKLE CELL DISEASE

A

HAEMOLYTIC

APLASTIC

SEQUESTRATION CRISIS

48
Q

HOW DO YOU DIAGNOSE NEONATAL JAUNDICE

A

MEASURE BILLIRUBIN

CHECK ANTENATAL BLOOD GROUPS, G6PD LEVELS AND FULL SEPTIC SCREEN

49
Q

WHAT IS SICKLE CELL DISEASE

A

A POINT MUTATION IN CODON 6 OF THE BETA GLOBIN GENE CAUSING HbS

50
Q

A PATIENT WITH HAEMOPHILLIA BLEEDS IN XS AFTER SURGERY WHAT IS THEIR SEVERITY OF HAEMOPHILLIA

A

MILD

51
Q

WHAT IS HAEMOPHILLIA

A

A GROUP OF COAGULATION DISORDERS WHICH ARE ‘COMMON’ AND INHERITED

52
Q

WHAT ARE THE TYPES OF SICKLE CELL DISEASE

A
  1. SICKLE CELL ANAEMIA
  2. HbSC DISEASE
  3. SICKLE BETA-THALASSEMIA
  4. SICKLE TRAIT
53
Q

WHAT ARE THE SIGNS OF ABO DISEASE

A

+VE COOMBS TEST

NO HEPATOSPLENOMEGALY

54
Q

WHAT IS THE PRESENTATION OF INTERMEDIA ALPHA THAMASSEMIA

A

MILD - MODERATE ANAEMIA

55
Q

WHAT IS FANCONI ANAEMIA

A

A RARE GENETIC DISORDER RESULTING IN IMPAIRED RESPONSE TO DNA DAMAGE

56
Q

WHAT IS A SIGN OF RHESUS INCOMPATIBILITY OF THE BABY

A

HEPATOSPLENOMEGALY

57
Q

WHAT IS THE DEFINITION OF ANAEMIA

A

Hb BELOW NORMAL RANGE

58
Q

WHAT IS IMMUNE THROMBOCYTOPENIA PURPURA

A

ISOLATED LOW PLATELET COUNT WITH A NORMAL BONE MARROW

59
Q

WHAT ARE CAUSES OF NEONATAL JAUNDICE WITHIN FIRST 24 HRS OF LIFE

A

HAEMOLYTIC DISORDERS

RHESUS

SBO

SPHEROCYTOSIS

60
Q

WHAT ARE THE TYPES OF BETA THAMASSEMIA

A

MAJOR (no Hb production)

INTERMEDIA (some HbA and some HbF)

TRAIT (asymptomatic)

61
Q

WHAT IS THE PRESENTATION OF A HAEMOLYTIC SICKLE CELL DISEASE ANAEMIC CRISIS

A

SOB

FATIGUE

PIAN

62
Q

WHAT ARE ABNORMAL CAUSES OF NEONATAL JAUNDICE WITHIN THE 24 HR-2W PERIOD

A

BREAST MILK JAUNDICE

INFECTIONS (UTI)

HAEMOLTIC DISORDERS

BRUISING

POLYCYTHEMIA

CRIGLE-NAJJAR

63
Q

WHAT FINDINGS ON FBC, AND IRON STUDIES WOULD YOU FIND FOR TRAI BETA THAMASSEMIA

A

HYPOCHROMIC

MICROCYTIC

NORMAL FERRATIN

64
Q

HOW WOULD YOU MANAGE A HAEMOPHILLIA CRISIS

A

IV FACTORS (8/9)

OR

OV HIGHLY PRUIFIED VIRALLY INACTIVE PLASMA DERIVED PRODUCTS

65
Q

DESCRIBE THE Hb COMPOSITION OF SICKLE BETA THALASSEMIA

A

HbS FROM ONE PARENT

Hb B THALLASSEMIA FROM OTHER

WILL ACT LIKE SICKLE CELL ANAEMIA (HbSS)

66
Q

A PATIENTS Hb COMES BACK THERE IS A MIX OF HbC AND SOME HbS WHAT SICKLE CELL DISEASE IS THIS

A

NORMALLY 60% HbC and 40% HbS

ASYMPTOMATIC

67
Q

WHAT IS THE Hb RANGE FOR 1-12M

(ANAEMIA)

A

<10

68
Q

IN FANCONI ANAEMIA WHAT ARE YOU AT RISK OF DEVELOPING

A

BINE MARROW FAILURE

AML

LYLEODYSPLASTIC SYNDROMES (PRE- LEUKAEMIA)

69
Q

WHAT IS THE PRESENTATION OF TRAIT ALPHA THAMASSEMIA

A

ASYMPTOMATIC

70
Q

WHAT POPULATIONS ARE COMMONLY AFFECTED BY BETA THAMASSEMIA

A

INDIAN

MIDDLE EASTERN

MEDITERRANIAN

71
Q

WHAT ARE THE THREE TYPES OF ALPHA THAMASSEMIA

A

MAJOR (ALL 4 GENES)

INTERMEDIA (3 GENES)

TRAIT (1-2 GENES)

72
Q

A PATIENT WITH A PMH OF SICKLE CELL DISEASE PRESENTS TO A + E WITH

  • DACTALYSIS
  • SWELLING
  • PAIN!!!

WHATS IS HER DIAGNOSIS

A

AN ACUTE CRISES OF SICKLE CELL DISEASE

  • HAND + FOOT SYNDROME
73
Q

WHAT ARE CAUSES OF APLASTIC ANAEMIA

A

CONGENITAL

TRANSIENT ERYTHROBLASTOPENIA

PARVOVIRUS + HEAMOLYTIC ANAEMIA / SICKLE CELL

74
Q

A PATIENT WITH HAEMOPHILLIA BLEEDS AFTER MINOR TRAUMA FOR A PROLONGED PERIOD OF TIME WHAT SEVERITY IS THIS

A

MODERATE

75
Q

WHAT IS THE TREATEMENT RHESUS INCOMPATIBILITY

A

ANTI-D IVIG

76
Q

WHAT IS KERNICTUS

A

AN ENCEPHALOPATHY RESULTING FROM DEPOSTITION OF UNCONJUGATED BILLIRUBIN IN BASAL GANGLIA AND BRAIN STEM NUCLEI

77
Q

WHAT ARE THE TYPES OF IMMUNE THROMBOCYTOPENIA PURPURA

A

ACUTE

  • POST INFECTIOUS LASTING <2M

CHRONIC

  • LONGER THAN SIX MONTHS
78
Q

WHAT ARE POSSIBLE COMPLICATIONS OF SICKLE CELL DISEASE

A

RECURRENT INFECTIONS - HiB/ PNEUMOCOCCUS

FEMORAL HEAD NECROSIS

SHORT STATURE

DELAYED PUBERTY

STROKE

CRADIAC ENLARGEMENT

RENAL DYSFUNCTION

79
Q

A PATIENTS Hb COMES BACK THERE IS A MIX OF HbS AND SOME HbF WHAT SICKLE CELL DISEASE IS THIS

A

HbSS

80
Q

WHAT ARE THE TYPES OF ANAEMIA

A

MICROCYTIC

NORMOCYTIC

MACROCYTIC

+CHROMICS

81
Q

HOW DO YOU TREAT IRON DEFICIENCY ANAEMIA

A

IRON SUPPLIMENTS

NIFEREX FOR 3 MONTHS AFTER IRON IS NORMAL

82
Q

HOW COMMON I SHAEMOLYTIC DISEASE OF THE NEWBORN

A

50%

83
Q

WHAT IS THE PRESENTATION OF KERNICTUS

A

POOR FEEDING

LETHARGY

HYPERTONIA

SEIZURES

COMA

84
Q

WHAT IS THE MANAGEMENT OF NEONATAL JAUNDICE

A

PHOTOTHERAPY

EXCHANGE TRANSFUSIONS

IVIG FOR RHESUS OR ABO DISEASE

85
Q

IS AN INFANT GETS KERNICTUS WHAT CAN OCCUR IN THE FUTURE AS A COMPLICATION

A

BRAIN DAMAGE

LEARNING DIFFICULTIES

CHOREOATHEROID CEREBRAL PAULSEY

86
Q

WHAT IS THE Hb RANGE FOR 1-12Y

(ANAEMIA)

A

<11

87
Q

WHAT ARE NEONATAL CONJUGATED JAUNDICE CAUSES AFTER 2 W

A

NEONATAL HEPATITIS SYNDROME

BILE DUCT OBSTRUCTION

88
Q

HOW CAN YOU CLASSIFY THE SEVERITY OF HAEMOPHILLIA

A

SEVERE = <1% FACTOR

MODERATE = 1-5% FACTOR

MILD 5-10% FACTOR

89
Q

HOW DO YOU MANAGE ACUTE CRISES OF SICKLE CELL DISEASE

A

FLUIDS

O2

ANALGESIA

CONSIDER AN EXCHANGE TRANSFUSION

Phase 3a (22 decks)

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