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Hematology > Anemias, Thalassemias, Hemophilia > Flashcards

Anemias, Thalassemias, Hemophilia Flashcards

1
Q

Management of haemophilia

A

Recombinant factor concentrate

Virally inactivated plasma products next

Desmopressin in Haem A

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2
Q

What does PT + APTT measure?

A

PT = factor 2, 5, 7, 10

APTT = factor 2, 5, 8, 9, 10, 11, 12 (intrinsic + extrinsic)

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3
Q

What is purpura fulminans?

A

Consequence of vasculitis in skin, can lead to necrosis

Happens after meningitis + VZV due to production of antiviral ab which react + inactivate protein S

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4
Q

What does purpura suggest?

A

Platelet <30

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5
Q

Diagnostic clues to haemolysis

A

Increased reticulocyte count = polychromasia (lilac colour) Raised urinary urobilinogen

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6
Q

S+S thalassaemia

A

Haemolysis leads to anaemia, hepato + splenomegaly

Increased levels of unconjugated bilirubin

Iron overload

Excess urinary urobilinogen

Frontal bossing, growth impairment

A thalaseemia major = usually fatal with hydrops fetalis

B thalassemia major = severe anemia developing during first year of life

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7
Q

Pathology of thalassaemia

A

Microcytic anaemia

Haemaglobinopathy that causes intrinsic abnormalities of RBC

Haemolysis due to absent HbA

a-thalassemia = caused by deletions in a-globin gene

b-thalassemia = caused by mutations in Bglobin gene

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8
Q

Complications of treatment for hemophilia

A

Inhibitors (ab) develop, reducing effectiveness of treatment

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9
Q

What are the causes of microcytic anemia?

A

Caused by inherited/ acquired anemias, small RBCs, varying Hb content

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10
Q

How are microcytic anemias diagnosed?

A

Diagnosed with CBC, peripheral blood smear, blood chemistry, iron studies

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11
Q

What are the types of microcytic anemia?

A

Fe deficiency, thalassemia, sideroblastic, chronic disease, lead poisoning (find those small cells last)

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12
Q

What are the iron study results for iron deficiency?

A

Low iron, low ferritin, high TIBC

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13
Q

What are the iron study results for lead poisoning?

A

Low iron, low ferritin, high TIBC

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14
Q

What are the iron study results for thalassemia?

A

Normal or increased iron + ferritin, normal TIBC

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15
Q

What is the pathology of iron deficiency anemia?

A

Microcytic, hypochromic anemia, small erythrocytes, decreased hemoglobin

▪Insufficient iron → decreased iron for hemoglobin synthesis → impaired erythropoiesis → production of microcytic, hypochromic erythrocytes

▫Insufficient iron to synthesize hemoglobin during erythropoiesis (most common cause of anemia worldwide)

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16
Q

What are the causes of iron deficiency?

A

Insufficient intake/absorption

▪Decreased intake (Eating disorders (e.g. pica, anorexia, bulimia); self-imposed dietary restrictions (e.g. vegan diet ))

▪Decreased absorption (Celiac disease, surgical resection of gastrointestinal (GI) tract,bariatric surgery, excessive dietary calcium)

Increased need: Pregnancy, lactation

Increased growth: Infants, children, adolescents

Overt blood loss: Hematemesis, trauma-related hemorrhage, heavy menses, hematuria, multiple blood donations

Occult blood loss: GI bleed (e.g. peptic ulcer, tumor); vascular lesions (e.g. hemorrhoids); hookworm/other helminthic infections

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17
Q

Complications of iron deficiency?

A

▪High-output heart failure, angina, cardiorespiratory failure

Infants, young children: Impaired growth, development

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18
Q

S+S iron deficiency

A

Pallor

Fatigue, activity intolerance, exertional dyspnea, angina

Compensatory mechanisms: Palpitations, increased pulse, increased cardiac output, tachypnea, selective shunting of blood to vital organs (e.g. skin to kidneys)

Effects on epithelial tissues: Glossitis, Cheilosis, Koilonychia, Esophageal stricture, Gastric atrophy, Blue sclera, Pagophagia (obsessive consumption of ice)

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19
Q

Lab results for iron deficiency

A

↓ red blood cell count

Low/normal reticulocytes

↓ hemoglobin, hematocrit

Hypochromic-microcytic erythrocytes

Decreased: mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC)

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20
Q

What is Plummer Vinson syndrome?

A

Plummer-Vinson syndrome, the triad of esophageal webs, atrophic glossitis, and iron deficiency anemia

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21
Q

Iron deficiency anemia Tx

A

Prescribe all people with iron deficiency anaemia oral ferrous sulfate 200 mg tablets two or three times a day — treatment should continue for 3 months after iron deficiency is corrected to allow stores to be replenished.

22
Q

When to refer pts with anemia?

A
  • Urgently refer people with iron deficiency anaemia using a suspected cancer pathway for an appointment within 2 weeks if they are:
    • Aged 60 years or over.
  • Consider an urgent referral for people with iron deficiency anaemia using a suspected cancer pathway for an appointment within 2 weeks if they are:
    • Aged under 50 years and present with rectal bleeding.
23
Q

S+S acute transfusion reaction

A

Fever, chills, pruritus, urticaria

Respiratory distres, haematuria, LOC, HTN/ hypotension, flank pain, jaundice, olgiuria

Disseminated bleeding/ bleeding from IV site

24
Q

Management of acute transfusion reaction

A

Stop transfusion

Maintain IV with saline

Confirm correct product was transfused, check product for color changes/ bubbles

Assess airway

Adrenaline for anaphylaxis, IV fluids if hypotensive, diuretics for transfusion associated circulatory overload

25
Q

Types of acute transfusion reaction

A

Fever/chills suggest an acute hemolytic transfusion reaction (AHTR), a septic transfusion reaction, transfusion-related acute lung injury (TRALI), or a febrile nonhemolytic transfusion reaction (FNHTR). Transfusion-associated circulatory overload (TACO) can cause resp distress.

26
Q

What is PTP + how is it treated?

A

Post transfusion purpura

IV immunoglobulins

27
Q

Causes of macrocytic anemia

A

Reticulocytosis - hemolytic anemia, bone marrow recovery after transplant, increased erythropoiesis after EPO

Megaloblastic anemia - B12/ folate deficiency, drug induced

Multifactoral - alcohol, liver disease, HIV, hypothyroidism, pregnancy, aplastic anemia, myeloma, bariatric surgery

28
Q

What meds cause macrocytic anemia?

A

Methotrexate, trimethoprim, fluorouracil, zidovudine, hydroxyurea

29
Q

What is pernicious anemia?

A

Autoimmune, failure to absorb B12 leading to macrocytic anemia

30
Q

What is the difference in symptoms between B12 + folate deficiency?

A

Neurologic symptoms (combined subacute degeneration) are seen in vitamin B12 deficiency but not in folate deficiency because of the role B12plays in fatty acid pathways

31
Q

What lab results will be seen in a healthy pt with severe blood loss?

A

Inceased MCV

32
Q

Management of B12 deficiency with + without neurological involvement

A

With neuro involvement = hydroxocobalamin 1mg IM on alternate days until no more improvement, then every 2 months

W/O neuro involvement = hydroxocobalamin 1mg IM 3 times a week for 2 weeks. Continue every 2 months if not diet related, if diet related advise taking cyanocobalamin 50-150mcg tablets daily or biannual hydroxocobalamin 1mg IM

33
Q

What food sources are high in B12?

A

Eggs, fortified products (soy, cereal, bread), meat, dairy, salmon, cod

34
Q

What x-ray finding in sickle cell anemia may also be seen in thalassemia?

A

The crew cut skull radiograph, due to bone marrow expansion, is seen in both conditions

35
Q

Causes of hemolytic anemia

A

Thrombotic microangiopathy (TMA) such as thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS)

Acute hemolytic transfusion reaction (AHTR)

Disseminated intravascular coagulation (DIC)

Clostridial sepsis

RBC parasite (eg, malaria, Babesia)

Drug-induced immune hemolysis

G6PD deficiency

Pyruvate kinase (PK) deficiency

Thalassemias

Sickle cell disease

Mechanical hemolysis from aortic stenosis or prosthetic heart valve

Osmotic lysis from hypotonic infusion

Paroxysmal nocturnal hemoglobinuria (PNH)

Hereditary spherocytosis (HS)

Hereditary elliptocytosis (HE)

Hereditary stomatocytosis (HSt)

Hereditary xerocytosis (HX)

36
Q

When is a diagnosis of hemolytic anemia suspected?

A

Pt with chronic or new onset symptoms of anemia, low Hb, and an increased reticulocyte count that is not explained by accelerated RBC production due to recent bleeding; repletion of iron, vitamin B12, folate, or copper; or administration of erythropoietin

●Signs of RBC destruction such as increased lactate dehydrogenase (LDH), low haptoglobin, increased unconjugated bilirubin.

37
Q

Management of hemolytic anemia

A

RBC transfusion if severe, active bleeding, symptoms of organ ischemia

Plasma exchange if TTP

Hydration (avoid transfusion) in acute hemolytic transfusion reaction - free Hb can cause renal failure, hypotension, DIC

38
Q

What are the initial presentations of haemophilia?

A

Easy bruising, forehead hematomas, hemarthrosis, bleeding orally, subgaleal or intracerebral haemorrhages

39
Q

Complications from bleeding in hemophilia

A

Neurologic sequelae of ICH

Joint destruction from repetitive hemarthosis

40
Q

How common are de novo mutations in hemophilia?

A

1/3 of hemophilia pts

41
Q

Investigations for hemophilia

A

Screening tests for hemostasis, factor activity levels, genetic testing

Factor level <40% of normal is diagnostic

42
Q

Investigations + diagnosis for thalassemia

A

FBC, blood smear, iron studies

Raised RBC, microcytic anemia, non-immune hemolysis

Thalassemia major - target cells + teardrop cells

Hb Barts + HbH = a thalassemia

Increased HbF + HbA2 = b thalassemia

DNA testing for definitive diagnosis

43
Q

What is aplastic anemia?

A

Due to infection, autoimmune disease or meds

Causes pancytopenia

Bone marrow is depleted

44
Q

What is Fanconi’s anemia?

A

Inherited defect in DNA repair causing aplastic anemia

45
Q

What 4 viral infections cause aplastic anemia?

A

Parvovirus B19

HIV

EBV

Viral hepatitis

46
Q

A patient presents with petechiae and bleeding from the gums, fatigue and pallor, and has had several recent infections; a bone marrow aspirate is dry making you suspect what process?Aplastic anemia; this patient has symptoms suggestive of thrombocytopenia, anemia, and neutropenia, which are confirmed by the hypocellular aspirate

A

Aplastic anemia; this patient has symptoms suggestive of thrombocytopenia, anemia, and neutropenia, which are confirmed by the hypocellular aspirate

47
Q

Why do patients with end-stage renal disease have normocytic, normochromic anemia?

A

The kidneys normally produce erythropoietin; in renal failure the loss of erythropoietin results in anemia, which is amenable to treatment with exogenous erythropoietin

48
Q

What are the S+S of B12 deficiency?

A

peripheral neuropathy, smooth tongue, angular stomatitis

49
Q

What is the Schilling test used for?

A

Pernicious anaemia diagnosis

50
Q

What is sickle cell a mutation of?

A

Glutamine to valine

Hematology (5 decks)

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