Anaemia/Hemolytic Anaemia Flashcards

1
Q

What do normal red cells look like?

A

Round with a central area of pallor approx. 1/3 the diameter of the cell; (Size usually compared to the nucleus of a normal small lymphocyte)

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2
Q

What are you assessing when examining RBCs on a blood film?

A

The size of the RBC, its shape, haemoglobin distribution and presence of inclusions.

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3
Q

What is Anaemia?

A
  • A reduction in the haemoglobin concentration of the blood
  • associated with a fall in red cell count or packed cell volume (PCV)
  • patients suffer from tissue hypoxia
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4
Q

In anaemias caused by hemolysis or hemorrhage the neutrophil and platelet counts are often raised. True or False

A

True

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5
Q

In infections and leukemias, the leucocyte count is often raised. True or False.

A

True

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6
Q

How is serum unconjugated bilirubin derived?

A

From breaking down hemoglobin

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7
Q

What is raised when ineffective erythropoiesis is marked?

A

Serum unconjugated bilirubin and lactate dehydrogenase (LDH).
note: reticulocyte count is low

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8
Q

How can you assess erythropoiesis (if its effective or not)?

A

By examining bone marrow, haemoglobin level and reticulocyte count.

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9
Q

Haemolytic anaemia is due to?

A

Shortened red blood cell survival and premature destruction.

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10
Q

Evidence of hemolysis/rbc destruction?

A
  • elevated reticulocyte count (that is not due to recent bleeding or recent iron or other nutrient replacement in nutrient deficiency states)
  • elevated unconjugated bilirubin
  • elevated LDH
  • elevated AST
  • decreased haptoglobin.
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11
Q

Aetiological classification of anaemia?

A
  • blood loss,
  • deficient erythropoiesis and
  • excessive hemolysis.
  • further subdivided into: hypo-regenerative and hyper-regenerative; these are the responses of the bone marrow.
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12
Q

There is a single way to diagnose hemolytic anaemia. T or F.

A

F- HA is only diagnosed if majority of tests are positive and anaemia is not due to another cause.

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13
Q

High reticulocyte count could indicate what?

A

Accelerated marrow production of RBC, bone marrow is increasing production to compensate for loss of rbcs; found in hemolytic anaemia.

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14
Q

Can there be hemolysis without reticulocytosis?

A
  • Yes, occurs when bone marrow is incapable of responding appropriately to anaemia.
  • iron, B12, folate or copper deficiency
  • alcohol
  • myelodysplastic syndrome (stem cells dont mature), aplastic anaemia (stem cells arent formed) or other bone marrow failure syndrome.
  • RBC aplasia from parvovirus infection (aplastic crises)
  • Drug induced marrow suppression eg chemotherapy
  • Lag in reticulocyte production in the first few days of new onset haemolysis.
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15
Q

Can there be hemolysis without anaemia?

A
  • Yes, occurs when bone marrow capacity to increase RBC production is sufficient to overcome the anaemia caused by hemolysis.
  • Despite normal Hb and Hct, haemolysis can still be detected from increased retic count, increased serum LDH, decreased serum haptoglobin and elevated red blood cell turnover from reticulocytes
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16
Q

Can there be reticulocytosis without hemolysis?

A

Duh,
- occurs during recovery from a bleeding episode,
- repletion of Fe, B12 or folate,
- administration of erythropoietin.
- Recovery from a bone marrow insult – Parvovirus, medication, alcohol

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17
Q

What’s Aplastic anemia?

A
  • Damage to your stem cells in your bone marrow causing a decrease in bone marrow production.
  • Leads to pancytopenia.
  • not the same as aplastic crises (caused by a parovrius)
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18
Q

what are the clinical features of congenital haemolytic anaemia?

A
  • Anaemia: may show pallor of mucous membranes
  • mild fluctuating jaundice
  • splenomegaly
  • no bilirubin in the urine but it may turn dark because of excess urobilinogen.
  • may have gall stones like in PK deficiency
  • may have aplastic crises: usually precipitated by infection with parvovirus which “switches off” erythropoiesis; decreased reticulocytes
  • may have leg ulcers like in HS & sickle cell
  • may have skeletal abnormalities like thalassemia & SCD
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19
Q

Describe Intravascular hemolysis. What are the main lab findings?

A

RBCs may be broken down directly in the circulation.
- Haemoglobinaemia (excess haemoglobin is blood stream)
- haemoglobinuria (excess haemoglobin in urine)
- haemosiderinuria (haemosiderin in the urine)
- methaemalbuminaemia (albumin and haem)

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20
Q

Describe Extravascular hemolysis. What are the features?

A
  • excess removal of red cells by the RE system like spleen macrophages.
  • features include: jaundice, gallstones, splenomegaly with raised reticulocytes, unonjugated bilirubin and absent haptoglobins.
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21
Q

What occurs in hereditary spherocytosis? Clinical features?

A
  • RBCs lose part of its membrane and become more spherical, and is ultimately prematurely destroyed.
  • pigment gallstones are frequent
  • may have aplastic crises
22
Q

Warm autoimmune hemolytic anaemia? lab findings? treatment?

A
  • RBCs lose part of its membrane and become more spherical, and is ultimately prematurely destroyed.
  • spleen is often enlarged, may occur alone or with other diseases like SLE or ITP.
  • lab findings: extravascular haemolytic anaemia with spherocytosis; DAT/ Coombs’ test is postive
  • treatment: remove underlying cause, corticosteroids (prednisolone), monoclonal Ab (rituximab), splenectomy
23
Q

Cold agglutinin disease? Clinical features? Lab findings? Treatment?

A
  • chronic hemolytic anaemia aggravated by the cold; IgM attaches to rbcs in the cold circulation (4 C)
  • ass. with intravascular hemolysis
  • mild jaundice and splenomegaly
  • lab findings: DAT reveals complement only on red cell surface, IgM wont be seen; spherocytosis is less marked; RBC agglutination in the cold
  • may transform into an aggressive lymphoma
  • treatment: alkylating agents (chlorambucil) and rituximab; note-corticosteroids are of no use.
24
Q

Septicaemia can cause intravascular hemolysis with marked spherocytosis. T or F.

A

T

25
Q

Malaria can cause extravascular hemolysis by destruction of parasitized red cells. T or F

A

T
note: it can also cause direct intravascular lysis.

26
Q

Acquired causes of hemolytic anaemia include?

A
  • warm (IgG) or cold (IgM) AIHA
  • allo-antibodies to rbcs (transfusions)
  • red cell fragmentation syndromes (MAHA)
  • infections (malaria)
  • toxins/chemicals/drugs (DIHA, DITMA)
  • physical agents (thermal injury)
  • paroxysmal nocturnal haemoglobinuria (acquired but has the intrinsic red cell membrane defect)
27
Q

Inherited/congenital causes of hemolytic anaemia include?

A
  • red cell membrane defects: eg hereditary spherocytosis, hereditary elliptocytosis, hereditary stomatocytosis
  • haemoglobin defects: eg sickle cell, thalassemia
  • enzyme deficiencies: eg G6PD deficiency, pyruvate kinase deficiency
28
Q

Disease conditions that involve haemolysis and thrombosis?

A
  • Paroxysmal nocturnal haemoglobinuria
  • Sickle cell
  • hereditary spherocytosis
  • autoimmune haemolytic anaemia (AIHA)
29
Q

Emergency management of hemolysis?

A
  • Blood transfusion
  • Plasma exchange
  • rate of HB decline
  • complement blockade
  • hydration and haemodynamic support
30
Q

The reticulocyte count provides a means of implicating either the marrow or the periphery as the source of anaemia. T or F.

A

T- whether or not the bone marrow is failing to make the red cells or trying to replace the red cells; assessing the response of the bone marrow to peripheral anaemia.

31
Q

An increase in 2,3-DPG is found in chronic anaemia. T or F.

A

T
note: this reduces oxygen affinity to Hb, therefore oxygen is delivered to the tissues more, and the oxygen curve shifts to the right.

32
Q

microcytic and hypochromic anaemia?

A
  • low mcv n low mch
  • egs iron deficiency and thalessemia
33
Q

normocytic and normochromic anaemia?

A
  • normal mcv n normal mch
  • egs acute blood loss, haemolysis, chronic disease, marrow infiltration
34
Q

macrocytic anaemia?

A
  • high mcv
  • eg megaloblastic anaemia
35
Q

Purple top tube contains what anticoagulant ?

A

EDTA- used for complete blood counts

36
Q

Blue top tube contains what anticoagulant?

A

Trisodium citrate- used for clotting

37
Q

Green top contains what anticoagulant?

A

Heparin- used for molecular studies, cytogenetics & WBC investigations

38
Q

normal range of Hb in men and women?

A
  • men: 13.5-17.5 g/dL
  • women: 11.5-15.5 g/dL
    note: infants- 13.5-19.5 g/dL
39
Q

whats hypo-regenerative anaemia?

A
  • failure of bone marrow production of red cells/ the problem is the bone marrow
  • RPI < 2/ low reticulocyte count
40
Q

whats hyper-regenerative anaemia?

A
  • increase in red cell destruction with a concomitant increase in red cell production/ problem is outside the bone marrow
  • RPI > 3/ high reticulocyte count
41
Q

whats the normal reticulocyte count in adults and children?

A

0.2-2.0%

42
Q

whats the normal MCV value in adults?

A

85 +/- 8 fl

43
Q

whats the normal MCH value in adults?

A

29.5 +/- 2.5 pg

44
Q

whats the normal MCHC value in adults and children?

A

33 +/- 2 g/dL

45
Q

An increase in red cell levels of 2,3 DPG is found in chronic anaemia. T or F.

A

T- This increase facilitates the delivery of oxygen to the tissues by reducing the affinity of haemoglobin for oxygen

46
Q

Most haemolytic anaemias are intravascular or extravascular?

A

extravascular ie in the spleen baby

47
Q

Differentiate between intrinsic and extrinsic causes of hemolytic anaemias?

A
  • intrinsic means there is a RBC membrane defect/ hereditary/ congenital
  • extrinsic means the environment is affecting the RBC/ acquired
48
Q

Which type of acquired H.A is the exception?

A

Paroxysmal nocturnal hemoglobinuria (PNH)- it’s an acquired but also intrinsic

49
Q

What are egs of immune acquired HA?

A
  • Warm AIHA
  • Cold agglutinin disease
  • Drug induced HA (DIHA)
  • Paroxysmal cold haemoglobinuria
50
Q

What are egs of non-immune acquired HA?

A
  • Microangiopathic hemolytic anaemia (MAHA)
  • Thrombotic thrombocytopenia purpura (TTP)
  • Haemolytic uraemic syndrome (HUS)
  • Drug induced thrombotic microangiopathy (DITMA)
  • Paroxysmal nocturnal haemoglobinuria (PNH)
51
Q

what are the clinical features of acquired haemolytic anaemia?

A
  • acute febrile illness
  • pallor or other features of anaemia
  • jaundice
  • may be insidious
  • features of underlying disease (lupus)
52
Q

A Coombs test does what?

A
  • Used for detection of Abs on patient’s RBC or in patient’s plasma.
  • Positive test means the patient has haemolysis
  • diagnoses AIHA