Haematology Flashcards by Claudia Zizzo

WHEN IN SICKLE CELL DISEASE WOULD YOU USE AN EXCHANGE TRANSFUSION

ACUTE CHEST SYNDROME

STROKE

PRIAPISM

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WHAT ARE NEONATAL UNCONJUGATED JAUNDICE CAUSES AFTER 2 W

BREAST MILK

HYPOTHYROIDISM

GI OBSTRUCTION IE PYLORIC STENOSIS

HAEMOLYTIC ANAEMIA

UTI/INFECTIONS

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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

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

HbSC

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WHAT IS THE PATHOPHYSIOLOGY OF RBC APLASIA

DECREASED RETICULOCYTES SO DECREASED LEVELS OF HB AND BILLIRUBIN

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WHAT ARE THE TREATEMENTS FOR FANCONI ANAEMIA

ANDROGENS

HAEMATIPETIC FACTORS

HAEMATOPOETIC STEM CELL TRANSPLANTS

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WHAT IS ALPHA THAMASSEMIA

A REDUCTION IN FUNCTIONING ALPA THAMASSEMIA

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WHAT IS THE MANAGEMENT OF ALPHA THAMASSEMIA

TREATED IN MAJOR:

MONTHLY BLOOD TRANSFUSTIONS

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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

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WHAT IS THE PRESENTATION OF FANCONI ANAEMIA

INFERTILITY (DECREASED SPERMATOGENESIS)

INCREASED PREDISPOSITION OF INFECTION

ANAEMIA SYMPTOMS

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WHAT IS THE PRESENTATION OF BETA THAMASSEMIA

PROLONGED NEONATAL JAUNDICE

SEVERE ANAEMIC

FAILURE TO TRIVE

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WHAT IS BETA THAMASSEMIA

SEVERE REDUCTION IN THE BETA GLOBIN GENE

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WHAT IS THE ONGOING MANAGEMENT FOR HAEMOPHILLIA PATIENTS

SEVERE: PROFYLACTIC IV FACTORS 8/9

MODERATE: DESMOPRESSIN

MILD: NOTHING

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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

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DESCRIBE WHAT CAN TRIGGER AN APLASITC CRISIS

PARVOVIRUS INFECTION CAUSING DECREASE IN RBC PRODUCTION

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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

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WHAT IS THE PRESENTATION OF SICKLE CELL DISEASE

ANAEMIA

JAUNDICE

VASOOCCULUSIVE CRISES’

ACUTE ANAEMIC CRISES’

SPLENOMEGALY

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WHEN IS RHESUS INCOMPATIBILITY DIAGNOSED

ANTENATALLY

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

SAME AS NORMAL PLUS

HYDROUREA (INCREASES HbF)

BONE MARROW TRANSPLANT

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WHAT IS THE Hb RANGE FOR NEONATES

(ANAEMIA)

UNDER 14

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WHAT ARE CAUSES OF MACROCYTIC ANAEMIA

ALCOHOL (NORMOBLASTIC)

FOLATE (MEGALOBLASTIC)

B12 (PERNACIOUS, MALABSORBTION AND TERMINAL ILEUM)

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WHAT IS THE PRESENTATION OF HAEMOPHILLIA

PROLONGED BLEEDING

BRUISING

SECURRENT SPONTANEOUS BLEEDS INTO JOINTS AND MUSCLES CAUSING PAIN

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WHAT ARE SIGNS OF FANCONI ANAEMIA

MICROCYTIC

PANCYTOPENIA

THROMBOCYTOPENIA

NEUTROPENIA

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WHAT ARE CAUSES OF MICROCYTIC ANAEMIA

IRON DEFICIENCY

THALASSEMIA

SICKLE CELL

CHRONIC DISEASE

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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

1. SICKLE CELL ANAEMIA 2. HbSC DISEASE 3. SICKLE BETA-THALASSEMIA 4. 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