Haematology Flashcards
WHEN IN SICKLE CELL DISEASE WOULD YOU USE AN EXCHANGE TRANSFUSION
ACUTE CHEST SYNDROME
STROKE
PRIAPISM
WHAT ARE NEONATAL UNCONJUGATED JAUNDICE CAUSES AFTER 2 W
BREAST MILK
HYPOTHYROIDISM
GI OBSTRUCTION IE PYLORIC STENOSIS
HAEMOLYTIC ANAEMIA
UTI/INFECTIONS
WHAT IS ABO?
WHEN MUM IS BLOOD GROUP O
BABY IS BLOOD GROUP A OR B
MATERNAL ANTIBODIES ATTACK BABYS Hb (ANTI A /ANTI B)
CAUSING HAEMOLYSIS
A PATIENT HAS A Hb COMPOSITION OF HbS AND HbC (although mostly abnormal) WHAT IS THEIR TYPE OF SICKLE CELL DISEASE
HbSC
WHAT IS THE PATHOPHYSIOLOGY OF RBC APLASIA
DECREASED RETICULOCYTES SO DECREASED LEVELS OF HB AND BILLIRUBIN
WHAT ARE THE TREATEMENTS FOR FANCONI ANAEMIA
ANDROGENS
HAEMATIPETIC FACTORS
HAEMATOPOETIC STEM CELL TRANSPLANTS
WHAT IS ALPHA THAMASSEMIA
A REDUCTION IN FUNCTIONING ALPA THAMASSEMIA
WHAT IS THE MANAGEMENT OF ALPHA THAMASSEMIA
TREATED IN MAJOR:
MONTHLY BLOOD TRANSFUSTIONS
HOW DO YOU DIAGNOSE IMMUNE THROMBOCYTOPENIA PURPURA
BLOODS: LOW PLATETLETS,
EXCLUDE:
HIV, CULTURES FOR MENINGITIS, ESR+CRP (SLE), ?BONE MARROW ASPIRATE TO EXCLUDE LEUKEMIA OR BLOOD FILM TO LOOK FOR BLASTOCYSTS
WHAT IS THE PRESENTATION OF FANCONI ANAEMIA
INFERTILITY (DECREASED SPERMATOGENESIS)
INCREASED PREDISPOSITION OF INFECTION
ANAEMIA SYMPTOMS
WHAT IS THE PRESENTATION OF BETA THAMASSEMIA
PROLONGED NEONATAL JAUNDICE
SEVERE ANAEMIC
FAILURE TO TRIVE
WHAT IS BETA THAMASSEMIA
SEVERE REDUCTION IN THE BETA GLOBIN GENE
WHAT IS THE ONGOING MANAGEMENT FOR HAEMOPHILLIA PATIENTS
SEVERE: PROFYLACTIC IV FACTORS 8/9
MODERATE: DESMOPRESSIN
MILD: NOTHING
WHAT IS THE PURPOUS OF VON WILLIBRAND FACTOR
FACILITATES PLATELET ADHESION TO ENDOTHELIUM WHEN DAMAGED ACTING AS A CARRIER FOR CLOTTING FACTORS (8)SO THAT THEY ARENT INACTIVATED AND THEN CLEARED
DESCRIBE WHAT CAN TRIGGER AN APLASITC CRISIS
PARVOVIRUS INFECTION CAUSING DECREASE IN RBC PRODUCTION
WHAT IS THE MANAGEMENT OF SICKLE CELL DISEASE
PROPHYLAXIS: FULL IMMS, DAILY PENICILLIN
DAILY FOLIC ACID
AVOIDING TRIGGERS (COLD, DEHYDRATION)
REGULAR OPHALMOGY CHECKS FOR RETINOPATHY
WHAT IS THE PRESENTATION OF SICKLE CELL DISEASE
ANAEMIA
JAUNDICE
VASOOCCULUSIVE CRISES’
ACUTE ANAEMIC CRISES’
SPLENOMEGALY
WHEN IS RHESUS INCOMPATIBILITY DIAGNOSED
ANTENATALLY
IN PATIENTS WHO HAVE FROQUENCT SICKLE CELL DISEASE CRISES WHAT IS THEIR MANAGEMENT
SAME AS NORMAL PLUS
HYDROUREA (INCREASES HbF)
BONE MARROW TRANSPLANT
WHAT IS THE Hb RANGE FOR NEONATES
(ANAEMIA)
UNDER 14
WHAT ARE CAUSES OF MACROCYTIC ANAEMIA
ALCOHOL (NORMOBLASTIC)
FOLATE (MEGALOBLASTIC)
B12 (PERNACIOUS, MALABSORBTION AND TERMINAL ILEUM)
WHAT IS THE PRESENTATION OF HAEMOPHILLIA
PROLONGED BLEEDING
BRUISING
SECURRENT SPONTANEOUS BLEEDS INTO JOINTS AND MUSCLES CAUSING PAIN
WHAT ARE SIGNS OF FANCONI ANAEMIA
MICROCYTIC
PANCYTOPENIA
THROMBOCYTOPENIA
NEUTROPENIA
WHAT ARE CAUSES OF MICROCYTIC ANAEMIA
IRON DEFICIENCY
THALASSEMIA
SICKLE CELL
CHRONIC DISEASE
WHAT ARE CAUSES OF NORMOCYTIC ANAEMIA
PREGNANCY
HAEMOLYTIC ANAEMIAS
APLASTIC
BLOOD LOSS
CHRONIC DISEASE
WHAT IS VON WILLIBRAND DISEASE
AN AUTOSOMAL DOMINANT DISEASE CAUSING DEFICIENCY OF VON WILLIBRAND FACTOR
WHAT ARE THE TWO TYPES OF HAEMOPHILLIA
ALPHA (FACTOR 8 DEFICIENCY)
BETA (FACTOR 9 DEFICIENCY)
WHAT IS THE TREATEMENT FOR KERNICTUS
PHOTOTHERAPY
BLOOD EXCHANGE TRANSFUSIONS
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
ACUTE CRISIS OF SICKLE CELL DISEASE
- ACUTE CHEST SYNDROME
WHAT IS THE PRESENTATION OF MAJOR ALPHA THAMASSEMIA
OEDEMATUS AND ASCITIS BABY ON 3RD TRIMESTER US
FATAL SHORTLY AFTER BIRTH IN MOST CASES
WHAT SIMPTOM OF HAEMOPHILLIA WOULD CLASSIFY THE HAEMOPHILLIA AS SEVERE
SPONTANEOUS BLEEDS INTO JOITS AND MUSCLES
WHAT SHOULD YOU INVESTIGATE FOR IF IRON DEFICIENCY ANAEMIA IS UNRESPONSIVE TO TREATEMENT
INVESTIGATE FOR COELIAC, MECKELS DIVERTICULUM, GI BLEEDS,OBG CAUSES
WHAT CAN GIVING IV CLOTTING FACTORS CAUSE AS A COMPLIATION
INFECTIONS
DAMAGED PERIFERAL ACCESS
DEVELOPING ANTIBODIES TO FACTORS 8/9
WHAT IS THE MANAGEMENT OF BETA THAMASSEMIA
MAJOR AND INTERMEDIA:
MONTHLY BLOOD TRANSFUSIONS
DESFERROXAMINE
BONE MARROW TRANSPLANT
TRAIT:
TREAT AS ABOVE BUT ONLY IF FERRATIN IS LOW
WHEN IS NEONATAL JAUNDICE PHYSIOLOGICAL
24 H - 2 W
WHAT IS CRAIG-NAJJAR
THERE IS NO GLUCURONILL TRANSFERASE CAUSING ANINCREASE IN UNCONGUGATED BILLIRUBIN
WHAT IS THE PRESENTATION OF IMMUNE THROMBOCYTOPENIA PURPURA
RED/PURPULE OURPURIC RASH AKIN TO THAT OF MENINGITIS
INCREASED TENDANCY TO BLEED
PETICHEAE
WHAT WOULD OCCUR IF BETA THAMASSEMIA WAS LEFT UNTREATED
EXTRAMEDULLARY HAEMOPOESIS (MARROW OVERGROWTH)
CAUSING
- MAXILLARY OVERGROWTH
- SKULL BOSSING
- HEPATOSPLENOMEGALY
WHAT INVESTIGAIONS ARE REQUIRED IN FANCONI ANAEMIA
FBC
MARROW ASPIRATE
GENETIC TESTING
HOW IS SICKLE CELL DISEASE SCREENED FOR
GUTHRIES TEST (HEEL PRICK)
IN HIGH RISK GROUPS CHORIONIC VILLUS SAMPLING
WHAT IS THE MANAGEMENT OF IMMUNE THROMBOCYTOPENIA PURPURA
STEROIDS (IV/PO)
AZOTHIOPRINE (IMMUNOSUPPRESSANTS)
ROMIPLOSTIM (THROMBOPOETIN RECEPTOR AGONIST TO STIMULATE PLATELET PRODUCTION)
PLATELET TRANSFUSIONS
SPLENECTOMY
HOW DO YOU TREAT RBC APLASIA
BLOOD TRANSFUSIONS
STEROIDS
SPLENECTOMY
WHAT IS HAEMOLYTIC DISEASE OF THE NEW BORN
HBF BEAKS DOWN FASTER THAN HBA CAN REPLACE SO BILLIRUBIN INCREASES
WHAT ARE COMPLICATIONS OF THE SPONTANEOUS BLEEDS INTO JOINTS AND MUSCLES
ARTHERITIS
WHAT INVESTIGATIONS ARE REQUIRED TO DIAGNOSE HAEMOPHILLIA
CLOTTING SCREEN LOOKING ESP AT FACTORS 8 + 9
WHAT CAN CAUSE A SEQUESTRATION CRISIS IN SICKLE CELL DISEASE
ACCUMULATION OF RBC IN SPLEEN
WHAT ARE THE THREE TYPES OF ACUTE ANAEMIC CRISIS IN SICKLE CELL DISEASE
HAEMOLYTIC
APLASTIC
SEQUESTRATION CRISIS
HOW DO YOU DIAGNOSE NEONATAL JAUNDICE
MEASURE BILLIRUBIN
CHECK ANTENATAL BLOOD GROUPS, G6PD LEVELS AND FULL SEPTIC SCREEN
WHAT IS SICKLE CELL DISEASE
A POINT MUTATION IN CODON 6 OF THE BETA GLOBIN GENE CAUSING HbS
A PATIENT WITH HAEMOPHILLIA BLEEDS IN XS AFTER SURGERY WHAT IS THEIR SEVERITY OF HAEMOPHILLIA
MILD
WHAT IS HAEMOPHILLIA
A GROUP OF COAGULATION DISORDERS WHICH ARE ‘COMMON’ AND INHERITED
WHAT ARE THE TYPES OF SICKLE CELL DISEASE
- SICKLE CELL ANAEMIA
- HbSC DISEASE
- SICKLE BETA-THALASSEMIA
- SICKLE TRAIT
WHAT ARE THE SIGNS OF ABO DISEASE
+VE COOMBS TEST
NO HEPATOSPLENOMEGALY
WHAT IS THE PRESENTATION OF INTERMEDIA ALPHA THAMASSEMIA
MILD - MODERATE ANAEMIA
WHAT IS FANCONI ANAEMIA
A RARE GENETIC DISORDER RESULTING IN IMPAIRED RESPONSE TO DNA DAMAGE
WHAT IS A SIGN OF RHESUS INCOMPATIBILITY OF THE BABY
HEPATOSPLENOMEGALY
WHAT IS THE DEFINITION OF ANAEMIA
Hb BELOW NORMAL RANGE
WHAT IS IMMUNE THROMBOCYTOPENIA PURPURA
ISOLATED LOW PLATELET COUNT WITH A NORMAL BONE MARROW
WHAT ARE CAUSES OF NEONATAL JAUNDICE WITHIN FIRST 24 HRS OF LIFE
HAEMOLYTIC DISORDERS
RHESUS
SBO
SPHEROCYTOSIS
WHAT ARE THE TYPES OF BETA THAMASSEMIA
MAJOR (no Hb production)
INTERMEDIA (some HbA and some HbF)
TRAIT (asymptomatic)
WHAT IS THE PRESENTATION OF A HAEMOLYTIC SICKLE CELL DISEASE ANAEMIC CRISIS
SOB
FATIGUE
PIAN
WHAT ARE ABNORMAL CAUSES OF NEONATAL JAUNDICE WITHIN THE 24 HR-2W PERIOD
BREAST MILK JAUNDICE
INFECTIONS (UTI)
HAEMOLTIC DISORDERS
BRUISING
POLYCYTHEMIA
CRIGLE-NAJJAR
WHAT FINDINGS ON FBC, AND IRON STUDIES WOULD YOU FIND FOR TRAI BETA THAMASSEMIA
HYPOCHROMIC
MICROCYTIC
NORMAL FERRATIN
HOW WOULD YOU MANAGE A HAEMOPHILLIA CRISIS
IV FACTORS (8/9)
OR
OV HIGHLY PRUIFIED VIRALLY INACTIVE PLASMA DERIVED PRODUCTS
DESCRIBE THE Hb COMPOSITION OF SICKLE BETA THALASSEMIA
HbS FROM ONE PARENT
Hb B THALLASSEMIA FROM OTHER
WILL ACT LIKE SICKLE CELL ANAEMIA (HbSS)
A PATIENTS Hb COMES BACK THERE IS A MIX OF HbC AND SOME HbS WHAT SICKLE CELL DISEASE IS THIS
NORMALLY 60% HbC and 40% HbS
ASYMPTOMATIC
WHAT IS THE Hb RANGE FOR 1-12M
(ANAEMIA)
<10
IN FANCONI ANAEMIA WHAT ARE YOU AT RISK OF DEVELOPING
BINE MARROW FAILURE
AML
LYLEODYSPLASTIC SYNDROMES (PRE- LEUKAEMIA)
WHAT IS THE PRESENTATION OF TRAIT ALPHA THAMASSEMIA
ASYMPTOMATIC
WHAT POPULATIONS ARE COMMONLY AFFECTED BY BETA THAMASSEMIA
INDIAN
MIDDLE EASTERN
MEDITERRANIAN
WHAT ARE THE THREE TYPES OF ALPHA THAMASSEMIA
MAJOR (ALL 4 GENES)
INTERMEDIA (3 GENES)
TRAIT (1-2 GENES)
A PATIENT WITH A PMH OF SICKLE CELL DISEASE PRESENTS TO A + E WITH
- DACTALYSIS
- SWELLING
- PAIN!!!
WHATS IS HER DIAGNOSIS
AN ACUTE CRISES OF SICKLE CELL DISEASE
- HAND + FOOT SYNDROME
WHAT ARE CAUSES OF APLASTIC ANAEMIA
CONGENITAL
TRANSIENT ERYTHROBLASTOPENIA
PARVOVIRUS + HEAMOLYTIC ANAEMIA / SICKLE CELL
A PATIENT WITH HAEMOPHILLIA BLEEDS AFTER MINOR TRAUMA FOR A PROLONGED PERIOD OF TIME WHAT SEVERITY IS THIS
MODERATE
WHAT IS THE TREATEMENT RHESUS INCOMPATIBILITY
ANTI-D IVIG
WHAT IS KERNICTUS
AN ENCEPHALOPATHY RESULTING FROM DEPOSTITION OF UNCONJUGATED BILLIRUBIN IN BASAL GANGLIA AND BRAIN STEM NUCLEI
WHAT ARE THE TYPES OF IMMUNE THROMBOCYTOPENIA PURPURA
ACUTE
- POST INFECTIOUS LASTING <2M
CHRONIC
- LONGER THAN SIX MONTHS
WHAT ARE POSSIBLE COMPLICATIONS OF SICKLE CELL DISEASE
RECURRENT INFECTIONS - HiB/ PNEUMOCOCCUS
FEMORAL HEAD NECROSIS
SHORT STATURE
DELAYED PUBERTY
STROKE
CRADIAC ENLARGEMENT
RENAL DYSFUNCTION
A PATIENTS Hb COMES BACK THERE IS A MIX OF HbS AND SOME HbF WHAT SICKLE CELL DISEASE IS THIS
HbSS
WHAT ARE THE TYPES OF ANAEMIA
MICROCYTIC
NORMOCYTIC
MACROCYTIC
+CHROMICS
HOW DO YOU TREAT IRON DEFICIENCY ANAEMIA
IRON SUPPLIMENTS
NIFEREX FOR 3 MONTHS AFTER IRON IS NORMAL
HOW COMMON I SHAEMOLYTIC DISEASE OF THE NEWBORN
50%
WHAT IS THE PRESENTATION OF KERNICTUS
POOR FEEDING
LETHARGY
HYPERTONIA
SEIZURES
COMA
WHAT IS THE MANAGEMENT OF NEONATAL JAUNDICE
PHOTOTHERAPY
EXCHANGE TRANSFUSIONS
IVIG FOR RHESUS OR ABO DISEASE
IS AN INFANT GETS KERNICTUS WHAT CAN OCCUR IN THE FUTURE AS A COMPLICATION
BRAIN DAMAGE
LEARNING DIFFICULTIES
CHOREOATHEROID CEREBRAL PAULSEY
WHAT IS THE Hb RANGE FOR 1-12Y
(ANAEMIA)
<11
WHAT ARE NEONATAL CONJUGATED JAUNDICE CAUSES AFTER 2 W
NEONATAL HEPATITIS SYNDROME
BILE DUCT OBSTRUCTION
HOW CAN YOU CLASSIFY THE SEVERITY OF HAEMOPHILLIA
SEVERE = <1% FACTOR
MODERATE = 1-5% FACTOR
MILD 5-10% FACTOR
HOW DO YOU MANAGE ACUTE CRISES OF SICKLE CELL DISEASE
FLUIDS
O2
ANALGESIA
CONSIDER AN EXCHANGE TRANSFUSION
