Benign hematology Flashcards by Douglas Tremblay

Blood group that Parvo binds to

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Salmonella infection in SS manifestations

Osteomyelitis of the long bones

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Indications for HU in SSD

> = 3 VOC per year
Recurrent ACS
Stroke history

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Goal HbS for exchange

<30%

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Goal HbS for chronic exchange transfusion

<30%

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Preoperative simple transfusion goal in SS

Hgb > 10

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Cancer risk for SS trait

Renal medullary cancer

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Goal HbF for HU in SSD

20%

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Hemoglobin M pathophysiology

Mutation in heme pocket and form iron-phenolate resistant to reduction in divalent state

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Hemoglobin M methemoglobinemia range

15-30%

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Hemoglobin M prognosis

life expectancy unchanged

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Hemoglobin M clinical manifestations

Cyanosis not responding to methylene blue

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Oxygen dissociation curve shift in methemoglobin

To the left - non-methemoglobin in ferrous state and have increased affinity for oxygen

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Clinical manifestations of methemoglobinemia

Cyanosis with normal pO2

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Culprits for acquired methemoglobinemia (4)

Bactrim
dapsone
aniline dyes
rasburicase

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Congenital deficiency causing methemoglobinemia

NADH-cytochrome b5 reductase

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Congenital risk factors for methemoglobinemia (2)

G6PD deficiency

Pyruvate kinase deficiency

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HbCC disease manifestations (3)

Mild hemolytic anemia
splenomegaly
prone to gallstones

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HbSC disease manifestations (4)

Less VOC
More retinopathy
Priapism
Ischemic necrosis of bone

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How to distinguish Hb C from Hb A2

citric agar for electrophoresis

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Pyruvate kinase deficiency inheritance pattern

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Gene mutation for pyruvate kinase deficiency

PKLR

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Deficiencies with pyruvate kinase deficiency

pyruvate and ATP

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Symptoms of pyruvate kinase deficiency (4)

Splenomegaly
Gallstones
Jaundice
Leg ulcers

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Sickle beta+ Hb electrophoresis

HbS: moderate HbA: low HbF: modeate HbA2: moderate

Sickle beta0 Hb electrophoresis

HbS: high HbA: absent HbF: moderate

HbSS Hb electrophoresis

HbS: very high HbA: absent HbA2: very low HbF: moderate

Sickle-HbC electrophoresis

HbS: moderate HbC: moderate HbS: moderate HbF: mild

Direct Coombs in drug induced AIHA

IgG-. C3+

Phosphoglycerate kinase deficiency gene

PGK1

Phosphoglycerate kinase deficiency clinical manifestations

non-spherocytic hemolytic anemia

Spleen change after RBC transfusion in splenic sequestration crisis

Spleen shrinks

Treatment of splenic sequestration crisis in SSD

Careful transfusion

Treatment of pulmonary HTN in SSD

Complications of zinc over supplement

Copper deficiency and pancytopenua

ESA resistannce in ESRD patient cause

Secondary hyperparathyroidism

Manifestations of hereditary elliptocytosis

hemolysis

Most common Coombs for warm agglutinin AIHA

IgG+, C3+

Target of warm agglunin antibody

Rh epitope

MCV and MCHC in cold agglutination

Both elevated

Red cell antigen for cold agglutination disease

Where red cell destruction takes place in cold agglutination disease

Liver

Treatment of cold agglutinin disease

Treat underlying disease | Steroids and splenectomy not effective

Treatment of refractory idiopathic cold agglutinin disease

Rituximab

Urgent treatment of cold agglutinin disease

Plasmapharesis

Paroxysmal cold hemoglobinuria test

Donath-Landsteiner Ab

Mechanism of antibiotic associated AIHA

Hapten reaction

Mutations for hereditary spherocytosis (3)

Spectrin Ankyrin Band 3

CBC finding for hereditary spherocytosis

Elevated MCHC

Confirmatory test for hereditary spherocytosis

Eosin-5-maleimide (EMA) is low

Treatment of hereditary spherocytosis in Jehovah's witness

Splenectomy

Inheritance for hereditary elliptocytosis

Peripheral blood findings for lack of Kell Ab

Acanthrocytes

Coombs negative hemolytic anemia, absence of morphologic abnormalities cause

Pyruvate kinase deficiency

Inheritance of PK deficiency

X linked

Peripheral blood smear in G6PD deficiency

Heinz bodies and bite cells

Enzyme inhibited by lead poisoning

Pyramidine-5-nucleotidase

Drugs associated with interferon

Gemcitabine, bevacizumab Tacriolimus, cyclosporine Cocaine

Metformin vitamin deficiency

B12

Major complication of thalassemia intermedia

osteoporosis

High/normal B12 but deficiency syndrome in children

Transcobalamin II deficiency

Hereditary pyropoikilocytosis symptoms

Severe hemolytic anemia | Young age of onset

When to suspect methemoglobinemia

Cyanosis with normal pO2

Treatment of cold agglutinin secondary to mycoplasma

Azithromycin

Majeed syndrome clinical characteristics (3)

Recurrent osteomyelitis Sweet syndrome Dyserythropoietic anemia

Majeed syndrome mutation

LPIN2

Target of Donath-Landsteiner Ab in PCH

P antigen

Oral iron with less GI side effects

Ferric maltol

HbE/beta thalassemia clinical characteristics

South Asia | Mild anemia

Cause of INH induced anemia

δ-Aminolevulinate-synthase inhibition

HbS/hereditary persistence of fetal Hb symptoms

Asymptomatic

HbH clinical manifestations

Mild anemia | Iron overload

Hb Barts condition

Hydrop fetalis or HbH

Hb Barts oxygen affinity

Extremely high

HbH disease alpha globin genes

(--,--), (--,a)

Hb Constant Spring defect

Point mutation leading to elongation of alpha globin chain

Hb Constant Spring clinical manifestations

Severe anemia | Normocytic

Thal minor designation

Hb b/b+ or Hb b0/b

Thal intermediate designation

Hb b+/b+ or b0/b+

Deficient enzyme in congenital methemoglobinemia

Cytochrome b5 reductase

Gene responsible for alpha thal

ATRX

Abetalipoproteinemai RBC morphology

acanthrocytes

Urgent treatment for babesiosis

Exchange transfusion plus atovaquone+azithro

Clinical characteristics for HbE beta-0

Similar to beta-thal intermediate

RDW in alpha and beta thal

normal

Treatment of congential ringed sideroblasts

Vit B6 (pyridoxine)

Hereditary pyrimidine 5'-nucleotidase deficiency treatment

Splenectomy

Origin of red cell destruction in colg agglutinin disease

Liver

Hemoglobin H cause

loss of 3 alpha chains

Hb Constant Spring

HbH with 2 deletions and 1 mutation

Hb Constant Spring clinical manifestations

Severe anemia | Splenomegaly

Moderate hemopphilia levels

1-5%

Dose of FVIII for prophylaxis or treatment

50U/kg

Treatment of mild hemophilia with mild bleeding

aminocaproic acid

Level maintained for hemophilia and major surgery

Prior to surgery: 100% | After surgery: 50%

Level maintained for hemophilia prophylaxis

>1%

Number of Bethesda units for factor to not work

Treatment of Hem A with inhibitor (3)

Recombinant VIIIa Porcine VIII Prothrombin concentrate (FEIBA)

Prolonged PT and PTT in amyloidosis cause

Factor X inhibitor

Findings of aVWD

Normal Ag Decreased ristocetin Decreased multimers

Level for VW for epidural

50 IU/dL

Blood type with low vW

Treatment of bleeding in vWD Type 2B

vWF concentrate + platelets

Treatment of Factor X def bleeding

Factor X concentrate OR | PCC

Type I VWD inheritance

Type I VWD vs Type III VWD

Some VWF vs none

Heye's syndrome bleeding diathesis

Acquired Type IIA VWD

Type IIA VWD findings

Absent large multimers

Goal FVIII level for joint bleeding

peripheral - 50% | proximal - 100%

VWD Type IC mechanism

Increased clearance of VWF

Treatment of Type IC VWD

Humate (FVIII + VWF)

Treatment of Type I VWD during pregnancy

Monitor levels (will rise during pregnancy) VWF > 50 DDAVP soft contraindication because of uterine contractions Continue replacement for 3 weeks

Treatment of hemophilia A bleed on emicizumab

FVIII

Glanzmann's thrombocythemia defect

GPIIb/IIIa

INR to give Vit K in nonbleeding warfarin

Factor deficiency leading to elevated PT and PTT but normal thrombin time (2)

Factor V | Factor X

Use of reptilase time

Not sensitive to anticoagulants

Duration of anticoagulation for high risk superficial vein thrombosis

45 days

Clinical manifestations Factor V

Minor bleeding | Elevations in PT and PTT

Treatment of bleeding with Factor V deficiency and goal FV level

FFP | 20%

5M urea clot lysis assay positive disease

Factor XIII deficiency

HELLP vs severe pre-eclampsia timing

Third trimester (HELLP) second for severe preeclampsia

Prolonged thrombin time or reptilase time meaning

low and dysfunctional fibrinogen

VW Type IIB diagnosis

Binds platelets - increased risocetin activity

Treatment of bleeding in VW Type IIB

Humate P (VW replacement)

VW Type IIN diagnosis

Nothing to do with FVIII (severe reductions because of degrading of FVIII)

Inheritance for Type I VW

Inheritance for Type III VW

Use of DDAVP in VW - which types

Type I and IIA with mild procedures

Treatment of FII deficiency associated with LAC

Observation, can resolve

Location of May-Thurner related clot

Left iliac

Type IIM VW diagnosis

Multimers normal present but decreased

Strong APLS Ab for thrombosis

Least thrombogenic contraceptive

IUD

Inheritance of antithrombin deficiency

Deficiency associated with purpura fulminans

Protein C deficiency

Indications for extended post-surgical prophylaxis (4 weeks)

abdominal or pelvic surgery for cancer

management of pregnant homozygous FVL without family or personal VTE history

observation + antepartum enoxaparin

Treatment of APLS with prior fetal loss during subsequent pregnancy

enoxaparin + ASA

Brain mets that can bleed (4)

Thyroid RCC Chorio Melanoma

Betrixaban indication

Hospital VTE prophylaxis

Duration of AC for pregnancy induced VTE

6 weeks postpartum and for 3 months total duration

Indications for iron chelation in beta thal (3)

Ferritin > 1000 liver iron concentration > 3mg/g 20-25 transfusion

Juvenile hemochromatosis gene

HJV

H63D homozygous HH manifestations

biochemical but not clinical HH

Goal ferritin in HH

<100

Goal ferritin in chelation for iron overload

<1000

Test for porphyria

urine porphobilogen (PBG)

Target of Hemin

ALAS1 synthesis

Treatment to prevent neonatal hemochromatosis in pregnant woman

IVIG

Iron overload with normal transferrin sat and neurological symptom diagnosis

Aceruloplasminemia

Iron refractory iron deficiency anemia gene

TMPRSS6

Iron deposition in Kuppfer cells disease

Classical ferroportin disease

Iron deposition in Kuppfer cells disease gene

FPN

Only HH gene that is AD

FPN1

Management of porphyria cutenea tarda

phlebotomy

Only porphyria inherited as AR

δ-ALA dehydratase porphyria

Treatment of porphyria cutaneous tarda refractory to phlebotomy

Hydroxychloroquine

Treatment of acute hepatic porphyria

Givosiran

Defect in Glazmann's thrombocythemia

integrin alpha2beta3

Defect in Bernard Soulier

GP Ib/IIa

Platelets in Bernard Soulier

Thrombocytopenia, large platelets

Findings on platelet aggregation studies for storage disease

Decreased aggregation in response to ADP, collagen, epinephrine

Hermansky Pudlack symptoms (3)

1) oculocutaneous albinism 2) bleeding diathesis 3) pulmonary fibrorsis

GP Ib/IX/V function

binds platelet to endothelium

Upshaw–Schulman deficiency

ADAMTS13

Clopidogrel hematology disease association

TTP

Quebec Platelet disorder deficiency

Urokinase plasminogen activator

Treatment of bleeding in Glanzmann's disorder 1st and 2nd line

1) platelets | 2) Recombinant FVIIa

Target of antibody in post-transfusion purpura

HPA-1a

MYH9-related disorders manifestations (3)

Thrombocytopenia Dohle-like bodies Sensorineal hearing loss

Glanzmann's thrombocythemia platelet aggregation study findinbg

Aggregate to risocetin, but not to ADP, collagen, epinephrine

Treatment of fetal and neonatal alloimmune thrombocytopenia

IVIG in mother at 20 weeks

Factors in cryopreciptate (4)

fibrinogen FVIII FXIII vWF

Most common virus acquired from transfusion

HBV

Management of AHTR (2)

Hydration | Alkaline urine

Treatment of neonate with alloimmune thrombocytopenia

Washed platelets from mother

Most common blood group antigens outside of ABO and Rh

Kell

Major mismatch situation

Recipient has antibodies to donor

Minor mismath situation

Donor has antibodies to recipient

Bidirectional situation

Both recipient antibodies to donor and donor antibodies to recipient

Treatment of cyclic neutrophilia in children

GCSF

Severe congenital neutropenia gene

ELANE

Chediak-Higashi morphology

Neutrophils with large lysosome inclusions

Test for CGD (2)

NBT or DHR

Treatment of CGD

Interferon gamma-1b

Physical findings in Fanconi anemia (3)

Microcephaly Skin hyperpigmentation Short stature

First line treatment for AA (non transplant)

Horse ATG, cyclosporine, +/- eltrombopag

Treatment of chronic parvovirus causing pure red cell aplasia

IVIG

Barth syndrome symptoms (3)

neutropenia dilated cardiomyopathy skeletal myopathy

WHIM syndrome gene defect

CXCR4

WHIM syndrome treatment

Plerixifor

When to treat PNH clone in AA

>50%

Gene associated with dyskeratosis congenita (2)

TERT | TERC

Gene defect in Diamond-Blackfan

RPS19

Pancreatic exocrine malfunction with neutropenia disease

Shwachman-Diamond syndrome

RDW in AOCD

Normal

Most common three alloantibodies to RBC

Kell, C, and E

Who to give postsplenectomy ppx to

age <16 or >50

AC for pregnant woman with provoked VTE and any thrombophilia

Antepartum and postpartum AC

AC for pregnant woman with APLS

Lovenox 40 and ASA 81

Treatment of post-transfusion purpura

IVIG

AC for pregnant woman with heterozygous FVL

Observation

% febrile neutropenia where you give GCSF

20%

Mechanism of iron overload in ineffective erythropoiesis

Increased erythroferrone production by erythroblasts

Deferasirox monitoring (3)

CMP (Cr and LFTs) Opthalmologic exam (annual) Auditory (annual)

Most common genotype for clinical HH

C282Y homozygous

Treatment of SS priapism

Urology evaluation

Back pain in Thal Major patient cause

EMH in spine

PK levels after splenectomy

Can be falsely low

Causes of secondary porphyria cutaneous tarda (2)

HIV | Alcohol

Indications for treatment of PNH

more than mild symptoms

Duration of AC ppx after cancer surgery

28 days

Postpartum cerebral sinus vein thrombosis associated with moderate hemorrhage

When to use procine rFVII

inhibitor level <20

Treatment of FVII deficiency

rFVIIa

Prolonged TT with prolonged PT and PTT

fibrinogen defect

Differentiating Glanzmans thrombothemia and Bernard Soulier

Bernard Soulier has thrombocytopenia

Platelet count in Glanzmans thrombothemia

Normal

Intraop AC in patient with hx of HITT but no PF4 Ab

Heparin

Diamond-Blackfan manifestation

Pancytopenia, family history of leukemia

Test for Diamond-Blackfan

erythrocyte adenosine deaminase concentration

Test for Fanconi anemia

Chromosome breakage analysis

Erdheim-Chester disease mutation

BRAF V600E

Type 2A VW findings

Absent large multimers

Target VW level for neuroaxial anesthesia

0.5-1.5

VW Activity:Ag ratio for Type 2

<0.7

Diagnosis of VWD levels

activity of antigen <30% or <50% with bleeding

VW Type that you cannot use desmopression

IIB

Response to risocetin Bernard Soulier vs Glanzmanns

Glansmanns aggregates | Bernard does not

Benign hematology Flashcards

(230 cards)