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UKMLA Prep > Clinical haematology > Flashcards

Clinical haematology Flashcards

Conditions and Presentations

1
Q

ALL

A
  • Most common form of malignancy which affects children
  • accounts for 80% of childhood leukaemia
  • peak is around 2-5 years old
  • boys affected more than girls are
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2
Q

ALL bone marrow failure signs

A

anaemia: lethargy and pallor
neutropaenia: frequent or severe infections
thrombocytopenia: easy bruising, petechiae

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3
Q

Other feautres of ALL

A

bone pain (secondary to bone marrow infiltration)
splenomegaly
hepatomegaly
fever is present in up to 50% of new cases (representing infection or constitutional symptom)
testicular swelling

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4
Q

Types of ALL

A

common ALL (75%), CD10 present, pre-B phenotype
T-cell ALL (20%)
B-cell ALL (5%)

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5
Q

Poor prognostic factors of ALL

A

age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex

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6
Q

Myeloma- CRABBI

A
  • calcium
  • Renal
    -Anaemia
    -Bleeding
    -Infetion
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7
Q

Myeloma calcium

A
  • hypercalcaemia

primary factor:
* increased osteoclastic bone resorption caused by local cytokines (e.g. IL-1, tumour necrosis factor) released by the myeloma cells
———————————————
much less common
* impaired renal function,
* increased renal tubular calcium reabsorption
* elevated PTH-rP levels

this leads to constipation, nausea, anorexia and confusion

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8
Q

Myeloma - Renal

A

Presentation:
* dehydration
* increased thirst

monoclonal production of immunoglobulins results in light chain deposition within the renal tubules

other causes of renal impairment in myeloma include
* amyloidosis
* nephrocalcinosis
* nephrolithiasis

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9
Q

Anaemia- myeloma

A

bone marrow crowding suppresses erythropoiesis leading to anaemia
this causes fatigue and pallor
* normocytic normochromic anemia

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10
Q

Bleeding myeloma

A

bone marrow crowding also results in thrombocytopenia which puts patients at increased risk of bleeding and bruising

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11
Q

Bones myeloma

A

bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity creates lytic bone lesions
this may present as pain (especially in the back) and increases the risk of pathological fractures

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12
Q

Myeloma infection

A

a reduction in the production of normal immunoglobulins results in increased susceptibility to infection

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13
Q

Other features of myeloma

A

amyloidosis e.g. macroglossia
carpal tunnel syndrome
neuropathy
hyperviscosity

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14
Q

Myeloma bloods investigation

A

full blood count: anaemia
peripheral blood film: rouleaux formation
urea and electrolytes: renal failure
bone profile: hypercalcaemia

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15
Q

Protein electophoresis investigation

A

raised concentrations of monoclonal IgA/IgG proteins will be present in the serum
in the urine, they are known as Bence Jones proteins

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16
Q

Major diagnostic criteria for Myeloma

A

Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine

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17
Q

Minor criteria of myeloma

A
  • 10% to 30% plasma cells in a bone marrow sample.
  • Minor elevations in the level of M protein in the blood or urine.
  • Osteolytic lesions (as demonstrated on imaging studies).
  • Low levels of antibodies (not produced by the cancer cells) in the blood.
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18
Q

Bruising in children - neonates

A

Coagulation disorders
haemorrhagic disease of the newborn
haemophilia

Thrombocytopaenia
maternal alloimmune thrombocytopaenia

Also
birth trauma: cephalohaematoma
congenital infections e.g. rubella

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19
Q

Infants bruising causes

A

Accidental injury

Non-accidental injury

Coagulation disorders
haemophilia

Thrombocytopaenia
ITP
Thrombocytopaenia with Absent Radius (TAR)
congenital infection

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20
Q

Older children bruising causes

A

Accidental injury

Non-accidental injury

Coagulation disorders
haemophilia
von Willebrand’s disease
liver disease

Thrombocytopaenia
ITP
acute lymphoblastic leukaemia
meningococcal septicaemia
Thrombocytopaenia with Absent Radius (TAR)
congenital infection

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21
Q

Common sites for bruises due to play

A

Shins
Elbows
Forehead

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22
Q

Bruises of concern in children.

A
  • excessive multiple bruises of different ages
    bruise patterns which may indicate slapping, being gripped tightly (fingertip marks) or the use of inflicting instruments (e.g. belt)
    sites which may raise concern include the face, ears, neck, buttocks, trunk or proximal parts of limbs
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23
Q

Normal colour changes in bruises

A

initially red
then changes to purple, blue or black (over 1-3 days)
later fades to yellow or green
light bruises typically fade within 2 weeks, more severe bruising may take longer

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24
Q

Haemophilia

A

X-linked recessive disorder of coagulation
- 30% of patients have no family history of the condition.

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25
Q

Hameophilia A

A

Deficiency of factor VIII

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26
Q

Christmas disease

A

Haemophilia B
Lack of factor IX

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27
Q

Features of haemophilia

A

haemoarthroses
haematomas
prolonged bleeding after surgery or trauma

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28
Q

Blood test findings in haemophilia

A

prolonged APTT
bleeding time, thrombin time, prothrombin time normal

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29
Q

Haemophilia A and antibodies

A

10-15% of patients with haemophilia A develop antibodies to factor VIII treatment.

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30
Q

Immune thrombocytopenia in children

A

ITP is am immune-mediated reduction in the platelet count
Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex. It is an example of a type II hypersensitivity reaction.
ITP more common acute in children than in adults, may follow an infection or vaccination

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31
Q

Features of ITP

A

bruising
petechial or purpuric rash
bleeding is less common and typically presents as epistaxis or gingival bleeding

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32
Q

Investigations of ITP

A

full blood count
should demonstrate an isolated thrombocytopenia
blood film
bone marrow examinations is only required if there are atypical features e.g.
lymph node enlargement/splenomegaly, high/low white cells
failure to resolve/respond to treatment

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33
Q

Managment of ITP

A

usually, no treatment is required
ITP resolves in around 80% of children with 6 months, with or without treatment
advice to avoid activities that may result in trauma (e.g. team sports)
other options may be indicated if the platelet count is very low (e.g. < 10 * 109/L) or there is significant bleeding. Options include:
oral/IV corticosteroid
IV immunoglobulins
platelet transfusions can be used in an emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by the circulating antibodies

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34
Q

Pernicious anaemia

A
  • autoimmune disorder which affects the gastric mucosa, results in vitamin B12 deficiency.
  • often delayed and subtle symptoms
  • causes can include H.pylori, alcoholism, gastrectomy…
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35
Q

Pathophysiology of pernicious anaemia

A

antibodies to intrinsic factor +/- gastric parietal cells
intrinsic factor antibodies → bind to intrinsic factor blocking the vitamin B12 binding site
gastric parietal cell antibodies → reduced acid production and atrophic gastritis. Reduced intrinsic factor production → reduced vitamin B12 absorption
vitamin B12 is important in both the production of blood cells and the myelination of nerves → megaloblastic anaemia and neuropathy

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36
Q

Risk factors of pernicious anaemia

A

more common in females (F:M = 1.6:1) and typically develops in middle to old age
associated with other autoimmune disorders: thyroid disease, type 1 diabetes mellitus, Addison’s, rheumatoid and vitiligo
more common if blood group A

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37
Q

Features of pernicious anaemia

A

anaemia features
lethargy
pallor
dyspnoea
neurological features
peripheral neuropathy: ‘pins and needles’, numbness. Typically symmetrical and affects the legs more than the arms
subacute combined degeneration of the spinal cord: progressive weakness, ataxia and paresthesias that may progress to spasticity and paraplegia
neuropsychiatric features: memory loss, poor concentration, confusion, depression, irritabiltiy
other features
mild jaundice: combined with pallor results in a ‘lemon tinge’
glossitis → sore tongue

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38
Q

Invesigations for pernicious anaemia

A

full blood count
macrocytic anaemia: macrocytosis may be absent in around of 30% of patients
hypersegmented polymorphs on blood film
low WCC and platelets may also be seen
vitamin B12 and folate levels
a vitamin B12 level of >= 200 nh/L is generally considered to be normal
antibodies
anti intrinsic factor antibodies: sensivity is only 50% but highly specific for pernicious anaemia (95-100%)
anti gastric parietal cell antibodies in 90% but low specificity so often not useful clinically
Schilling test is no longer routinely done
radiolabelled B12 given on two occasions, firstly on its own, secondly with oral IF. Urine B12 levels are then measured

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39
Q

Managment of pernicious anaemia

A

vitamin B12 replacement
usually given intramuscularly
no neurological features: 3 injections per week for 2 weeks followed by 3 monthly treatment of vitamin B12 injections
more frequent doses are given for patients with neurological features
there is some evidence that oral vitamin B12 may be effective for providing maintenance levels of vitamin B12 but it is not yet common practice
folic acid supplementation may also be required

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40
Q

Complications of pernicious anaemia

A

Increased risk of gastric cancer

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41
Q

Lymphadenopathy

A
  • enlarged lymph nodes.
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42
Q

Infective differential diagnosis for lymphadenopathy

A

infectious mononucleosis
HIV, including seroconversion illness
eczema with secondary infection
rubella
toxoplasmosis
CMV
tuberculosis
roseola infantum

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43
Q

Neoplastic causes of lymphadenopathy

A

leukaemia
lymphoma

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44
Q

Other causes of lymphadenopathy

A

autoimmune conditions: SLE, rheumatoid arthritis
graft versus host disease
sarcoidosis
drugs: phenytoin and to a lesser extent allopurinol, isoniazid

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45
Q

Massive haemorrhage definition

A

loss of one blood volume in a 24 hour period or the loss of 50% of the circulating blood volume in 3 hours.

A blood loss of 150ml/ minute is also included.

The normal adult blood volume is 7% of total adult body weight. The blood volume equates to between 8 and 9% of a child’s body weight.

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46
Q

Complications of massive haemorrhage- hypothermia

A

Blood is refrigerated
Hypothermic blood impairs homeostasis
Shifts Bohr curve to the left

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47
Q

Hypocalcaemia complications in haemorrhage

A

Both FFP and platelets contain citrate anticoagulant, this may chelate calcium

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48
Q

Hyperkalaemia massive haemorrhage risk

A

Plasma of red cells stored for 4-5 weeks contains 5-10 mmol K+

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49
Q

Delayed type transfusion

A

Due to minor incompatibility issues especially if urgent or non cross matched blood used

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50
Q

Transfusion related lung injury

A

Acute onset non cardiogenic pulmonary oedema
Leading cause of transfusion related deaths
Greatest risk posed with plasma components
Occurs as a result of leucocyte antibodies in transfused plasma
Aggregation and degranulation of leucocytes in lung tissue accounts for lung injury

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51
Q

Coagulopathy

A

Anticipate once circulating blood volume transfused
1 blood volume usually drops platelet count to 100 or less
1 blood volume will both dilute and not replace clotting factors
Fibrinogen concentration halves per 0.75 blood volume transfused

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52
Q

Common causes of hepatomegaly

A

-Cirrhosis: if early disease, later liver decreases in size. Associated with a non-tender, firm liver
-Malignancy: metastatic spread or primary hepatoma. Associated with a hard, irregular. liver edge
-Right heart failure: firm, smooth, tender liver edge. May be pulsatile

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53
Q

Causes of hepatomegaly

A

viral hepatitis
glandular fever
malaria
abscess: pyogenic, amoebic
hydatid disease
haematological malignancies
haemochromatosis
primary biliary cirrhosis
sarcoidosis, amyloidosis

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54
Q

Causes of hepatosplenomegaly

A

chronic liver diseasewith portal hypertension
infections: glandular fever, malaria, hepatitis
lymphoproliferative disorders
myeloproliferative disorders e.g. CML
amyloidosis

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55
Q

Massive splenomegaly causes

A

myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher’s syndrome

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56
Q

Other causes of splenomegaly

A

portal hypertension e.g. secondary to cirrhosis
lymphoproliferative disease e.g. CLL, Hodgkin’s
haemolytic anaemia
infection: hepatitis, glandular fever
infective endocarditis
sickle-cell*, thalassaemia
rheumatoid arthritis (Felty’s syndrome)

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57
Q

Fissure in and

A
  • Bright red rectal bleeding
  • painful bleeding that occurs post defecation, small volumes
  • associated with constipation
  • Muco-epithelial defect usually in the midline posteriorly (anterior fissures more likely to be due to underlying disease)
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58
Q

Haemorrhoids

A
  • bright red rectal bleeding
  • post dedication bleeding on toilet paper and in pan
  • altered bowel habit and history of straining
    -no blood mixed with stool
  • no local pain
  • colon and rectum normal
  • proctoscopy may show internal haemorrhoid, usually impalpable
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59
Q

Crohn’s disease

A
  • Bright red or mixed blood
  • bleeding may be accompanied by other symptoms such as altered bowel habit, malaise, history of tissues, assessed ‘
  • Perineal inspection may show fissures or fistulae. Proctoscopy may demonstrate indurated mucosa and possibly strictures. Skip lesions may be noted at colonoscopy.
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60
Q

Ulcerative colitis

A
  • bright red bleeding often mixed with stool
  • Diarrhoea, weight loss, nocturnal incontinence passage of mucous PR
  • Proctitis is most marked finding

Perianal disease is usually absent. Colonoscopy will show continuous mucosal lesion.

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61
Q

Rectal cancer

A

Bright red blood mixed volumes
- Alteration of bowel habit. Tenesmus may be present. Symptoms of metastatic disease.

Usually obvious mucosal abnormality. Lesion may be fixed or mobile depending upon disease extent. Surrounding mucosa often normal, although polyps may be present.

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62
Q

All patients rectal bleeding investigations

A
  • DRE
  • procto- sigmoidoscopy
  • young patients need require bowel preparation with an enema and a flexible sigmoidscopy performed.
    Patients with excessive pain and suspected of fissure can be given a GA or LAA
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63
Q

Fissure in ano managment

A

GTN ointment 0.2% or diltiazem cream applied topically is the usual first line treatment. Botulinum toxin for those who fail to respond. Internal sphincterotomy for those who fail with botox, can be considered at the botox stage in males.

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64
Q

Haemorrhoid managment

A

Lifestyle advice, for small internal haemorrhoids can consider injection sclerotherapy or rubber band ligation. For external haemorrhoids consider haemorrhoidectomy. Modern options include HALO procedure and stapled haemorrhoidectomy.

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65
Q

Inflammatory bowel disease managment

A

Medical management- although surgery may be needed for fistulating Crohns (setons).

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66
Q

Rectal cancer managment

A

Anterior resection or abdomino-perineal excision of the colon and rectum. Total mesorectal excision is now standard of care. Most resections below the peritoneal reflection will require defunctioning ileostomy. Most patients will require preoperative radiotherapy.

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67
Q

Disseminated intravasular coagulation

A
  • process of coagulation and fibrinolysis are dysregulated, results in widespread clotting and resulting bleeding.
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68
Q

Causes of DIC

A

sepsis
trauma
obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome)
malignancy

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69
Q

Diagnosis of DIC

A

platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia

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70
Q

Haemochromatosis features

A

early symptoms include fatigue, erectile dysfunction and arthralgia (often of the hands)
‘bronze’ skin pigmentation
diabetes mellitus
liver: stigmata of chronic liver disease, hepatomegaly, cirrhosis, hepatocellular deposition)
cardiac failure (2nd to dilated cardiomyopathy)
hypogonadism (2nd to cirrhosis and pituitary dysfunction - hypogonadotrophic hypogonadism)
arthritis (especially of the hands

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71
Q

Reversible complications of haemochromatosis

A

Cardiomyopathy
Skin pigmentation

72
Q

Irreversible complications of haemochromatosis

A

Liver cirrhosis**
Diabetes mellitus
Hypogonadotrophic hypogonadism
Arthropathy

73
Q

Haemochromatosis

A
  • autosomal recessive disorder of iron and absorption and metabolism results in iron accumulation
  • mutation of the HFE gene on both copies of chromosome 6
74
Q

Blood films

A

Manual examination of blood under microscope for abnormal shapes, sizes or inlclusions

75
Q

Anisocytosis

A

Variation in size of red blood cell
- may be seen in myelodysplatic syndrome

76
Q

Target

A

Red blood cells with central pigemented area surrounded by a pale area, surrounded by pale area.
Thicker cytoplasm on outside
Most commonly associated with iron deficiency anaemia and post-splenectomy

77
Q

Heinz bodies

A

Individual blobs called inclusions
Blobs are denatures haemoglobin
Usually seen in G6PD deficiency and alpha thalassameia

78
Q

Howell-jolly bodies

A

Howell-Jolly blobs of DNA material seen inside red blood cell
Spleen normally removes it
Commonly seen in patients with non-functioning spleens or severe anaemia

79
Q

Reticulocytes

A

Immature red blood cells
Slightly larger than normal red blood cells (erythrocytes)

80
Q

Schistocytes

A

fragments of red blood cells

81
Q

Microangiopathic haemolytic anaemia (MAHA

A

small blood clots (thrombi) obstruct small blood vessels.
Result in hameolysis

82
Q

Sideroblasts

A

immature red blood cells with a nucleus surrounded by iron blobs.

bone marrow cannot incorporate iron into the haemoglobin molecules.

Genetic defect or myelodysplastic syndrome

83
Q

Smudge cells

A

Associated with chronic lymphocytic leukaemia
Ruptured white blood cells

84
Q

Spherocytes

A

Sphere-shaped red blood cells that are bi-concave disc shaped

Associated with autoimmune haemolytic anaemia and hereditary spherocytosis

85
Q

Three types of anaemia

A

Microcytic anaemia (low MCV)
Normocytic anaemia (normal MCV)
Macrocytic anaemia (large MCV)

86
Q

Tails- microcytic anemia

A

T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia

87
Q

Normacytic anaemia- AAAHH

A

A – Acute blood loss
A – Anaemia of chronic disease
A – Aplastic anaemia
H – Haemolytic anaemia
H – Hypothyroidism

88
Q

Anaemia of chronic disease

A
  • chronic disease
  • chronic kidney disease due to reduced production of erythropoietin by the kidneys

Tx of erythropoietin

89
Q

Megaloblastic anaemia

A

B12 deficiency
Folate deficiency

90
Q

Noroblastic macrocytic anaemia

A

Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs, such as azathioprine

91
Q

Symptoms of anaemia

A

Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions, such as angina, heart failure or peripheral arterial disease

Pica
Hair loss

92
Q

Signs of anaemia

A

Pale skin
Conjunctival pallor
Tachycardia
Raised respiratory rate

93
Q
A

Koilonychia refers to spoon-shaped nails and can indicate iron deficiency anaemia
Angular cheilitis can indicate iron deficiency anaemia
Atrophic glossitis is a smooth tongue due to atrophy of the papillae and can indicate iron deficiency anaemia
Brittle hair and nails can indicate iron deficiency anaemia
Jaundice can indicate haemolytic anaemia
Bone deformities can indicate thalassaemia
Oedema, hypertension and excoriations on the skin can indicate chronic kidney disease

94
Q

Unexplained iron deficiency anaemia

A

Colonoscopy
OGD

Bone marrow biopsy `

95
Q

Causes of iron deficient anaemia

A

Insufficient dietary iron (e.g., restrictive diets)
Reduced iron absorption (e.g., coeliac disease)
Increased iron requirements (e.g., pregnancy)
Loss of iron through bleeding (e.g., from a peptic ulcer or bowel cancer)

96
Q

Bleeding in the GI tract

A

Cancer (e.g., stomach or bowel cancer)
Oesophagitis and gastritis
Peptic ulcers
Inflammatory bowel disease
Angiodysplasia (abnormal vessels in the wall)

97
Q

How to test for iron deficiency anaemia

A

TIBC which directly relates to ferretin

98
Q

ALL investigations

A
  • Leucocytosis on FBC
  • Blast cells on blood film and bone marrow
  • Lymphoblasts are typically large with nucleoli, with very little cytoplasm and no granularity
  • Immunophenotyping origin is a T or B cell, along with other immunological subtypes
  • Periodic acid–Schiff (PAS) stains for carbohydrate material in ALL
99
Q

When urgent FBC needed for leukaemia?

If there are any of the following

A
  • Pallor
  • Persistent fatigue
  • Unexplained fever
  • Unexplained persistent or recurrent infection
  • Generalized lymphadenopathy
  • Unexplained bruising
  • Unexplained bleeding
  • Unexplained petechiae
  • Hepatosplenomegaly
100
Q

Managment of ALL

A
  • combination chemotherapy
  • CNS prophylactic agents are given to all patients with ALL
  • Maintenace therapy for 2 years after
101
Q

How to monitor if ALL therapy is effective?

A
  • morphological remission is defined by blast count < 5%
  • Assessment of minimal residual disease (MRD) – greater sensitivity than morphological assessment

PCR to amplify the characteristic clonal rearrangements of:
* Immunoglobulin genes in B-cell ALL
* T-cell receptor genes in T-cell ALL
With the detection of 1 leukaemic cell in 10,000 cells in the bone marrow

102
Q

Complications of ALL

A
  • Increased risk of infection (use prophylactic antibiotics)
  • CNS involvment
  • Chemotherapy toxicity- mucositis, neutropenic fever, and cardiotoxicity.
103
Q

Side effects of Methotrexate

A
  • Mucositis
  • Myelosuppression
  • Pulmonary fibrosis
  • Liver fibrosis
104
Q

cautions of Methotrexate

A
  • photosensitivity
  • dehydration (increased risk of toxicity)
  • diarrhoea
  • peptic ulcer disease.
105
Q

Contradicting medications methotrexate

A

* trimethoprim or co-trimoxazole- bone marrow aplasia
* chemotherapeutic agents and **mono-clonal antibodies **increase risk of myelosuppression

106
Q

Monitoring required with Methotrexate?

A
  • Full blood count
  • renal function
  • liver function tests

done before starting treatment and repeated weekly until the patient is established on the drug. After this, they should be monitored every 2-3 months.

107
Q

causes of microcytic anaemia

A
  • Iron deficicinecy anaemia
  • Sideroblastic anemia
  • lead poisoning
  • Thalassemia
  • Iron loading anaemia
108
Q

Side effects of iron tablets

A

diarrhoea, constipation, black stools, abdominal pain, nausea

109
Q

Oral Iron and Levothyroxine

A
  • advise patients to take at least 4 hours apart.
  • iron reduces uptake of levo
110
Q

Signs and symptoms of microcytic anaemia

A
  • Fatigue and Weakness
  • Pallor
  • Shortness of Breath
  • Palpitations
  • Cold intolerance
  • Iron-deficiency anaemia specific:
  • Nail changes such as koilonychia (spoon-shaped nails)
  • Atrophic glossitis
  • Angular stomatitis
  • Pica
111
Q

Classification of VWD

A
  • Type 1 VWD: Partial quantitative deficiency in VWF
  • Type 2 VWD: Qualitative defects in VWF (e.g. decreased adhesion to platelets or factor VIII)
  • Type 3 VWD: Almost complete deficiency of VWF
112
Q

signs of VWD

A
  • Excess or prolonged bleeding from minor wounds
  • Excess or prolonged bleeding post-operatively
  • Easy bruising
  • Menorrhagia
  • Epistaxis
  • GI bleeding
113
Q

Investigation for VWD

A
  • Normal PT and TT
  • prolonged APTT (functional factor VIII deficiency) and bleeding time.
  • Von Willebrand factor level and activity assay
114
Q

Acute bleeding VWD managment

A
  • Desmopressin
  • Tranexamic acid can be given for minor bleeding or prior to surgery on its own or as an adjunctive therapy to desmopressin or concentrates
  • VWF-FVIII concentrates should be used if the above are unsuccessful and bleeding is persistent
115
Q

VWD which factor affected?

A

clotting factor VIII

116
Q

Acute emergency myeloma

A
  • Acute renal failure – swift treatment of volume depletion, early involvement of renal physicians
  • Hypercalcaemia – fluid and bisphosphonates needed
  • Hyperviscosity – plasmapheresis
  • Spinal cord compression – s radiotherapy emergency
117
Q

Haematopoietic stem cell transplantation

A
  • consider this option up to the age of 70 years
  • induction therapy
  • conditioning regimen (high-dose melphalan) followed by autologous stem cell transplan
118
Q

What is induction therapy

A
  • nonchemotherapeutic (eg. bortezomib, thalidomide and dexamethasone) (VTD) given together with Daratumumab (monoclonal antibody binding CD38) (DVTD).
  • **VTD with DVTD **
119
Q

Patients unsuitable for stem cell transplantation myeloma

A
  • melphalan, prednisolone and thalidomide (MPT)
  • patients likely to relapse after
  • supportive managment
120
Q

Complications of myeloma

A
  • Bone Disease
  • renal dysfunction
  • infection
  • anaemia
121
Q

factors 1 to 4

A
  • Factor I is fibrinogen.
  • Factor II is prothrombin.
  • Factor III is tissue factor (TF in the image above).
  • Factor IV is calcium. EDTA in purple blood tubes binds to calcium to prevent blood from clotting before it has been analysed.
122
Q

tumour lysis syndrome investigations

A
  • U&E: Potassium and phosphate are usually raised, raised Cr suggestive of AKI/renal failure.
  • Calcium: Typically low in tumour lysis syndrome.
  • Uric acid: Usually elevated.
  • ECG: To identify any metabolic abnormalities such as hyperkalaemia that may precipitate life-threatening arrhythmias.
123
Q

Managment of tumor lysis syndrome

A

~~~

```* Correction of electrolyte imbalances: This may require dialysis in severe cases.
* Administration of intravenous fluids: To help flush out the intracellular contents released by the dying tumour cells.
* Medications: Rasburicase

124
Q

Rasburicase mechanism

A

transforms uric acid into allantoin. Allantoin is more soluble in urine than uric acid, and more easily eliminated by the kidney.

125
Q

Signs and symptoms of tumour lysis syndrome

A
  • Dysuria or oliguria
  • Abdominal pain
  • Weakness
  • Nausea or vomiting
  • Muscle cramps
  • Seizures
  • Cardiac arrhythmias
  • Gout/joint swelling
126
Q
A
127
Q

Tumour lysis syndrome findings

A

increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure

128
Q

Cairo-Bishop scoring system

A

abnormality in two or more of the following, occurring within three days before or seven days after chemotherapy.
* uric acid > 475umol/l or 25% increase
* potassium > 6 mmol/l or 25% increase
* phosphate > 1.125mmol/l or 25% increase
* calcium < 1.75mmol/l or 25% decrease

129
Q

Electrolyte changes seen with transfusion

A
  • hypocalemia
  • hyperkalaemia
130
Q

AML

A
  • Malignancy
  • uncontrolled proliferation of myeloid precursors in the bone marrow
  • leading to bone marrow failure and the accumulation of immature white blood cells (blasts) in the peripheral blood.
131
Q

Epidemiology of AML

A
  • Primarily affects adults
  • Idiopathic
  • exposure to ionizing radiation
132
Q

Signs and symptoms of AML

A
  • Bone marrow failure – anaemia, bleeding, infections
  • Signs of tissue infiltration, including hepatomegaly, splenomegaly and gum hypertrophy
  • Central nervous system involvement is rare
  • Constitutional symptoms such as fever and unintentional weight loss
133
Q

Investigations of AML

A
  • blood tests (leucocytosis)
  • blood film- blasts
  • Blood marrow biopsy
  • hypercellular marrow
  • the presence of blasts (usually >50%)
  • Sometimes Auer rods
134
Q

NICE urgent FBC within 48hrs

A
  • Pallor
  • Persistent fatigue
  • Unexplained fever
  • Unexplained persistent or recurrent infection
  • Generalized lymphadenopathy
  • Unexplained bruising
  • Unexplained bleeding
  • Unexplained petechiae
  • Hepatosplenomegaly
135
Q

Managment of AML

A
  • chemotherapy (lasts 5-10 days)
  • Bone marrow transplant
  • Prophylactic antimicrobials
  • Blood products and growth hormone therapy
  • offer all crypopreservation
136
Q

AML prognosis

A
  • 3-year survival rate 20%
  • 30% cure rate with chemotherapy
137
Q

How is AML classified morphologically according to the French–American–British (FAB) classification?

A

AML is classified into eight subtypes (M0–M7).

138
Q

What does the FAB classification system for AML include?

A

The FAB classification system includes subtypes M0 to M7 based on morphological appearance.

139
Q

What is the role of Sudan Black in cytochemistry for AML?

A

Sudan Black stains lipid material in myoblasts.

140
Q

Which cytochemical stain is used to identify monocytic variants in AML?

A

Nonspecific esterase is used, which stains monocytic variants in AML (M5)

141
Q

What are common tissue infiltration sites for AML?

A
  • skin
  • liver
  • gums.
142
Q

How does the release of lysozyme from malignant cells affect AML patients?

A
  • damange to the renal tubules
  • potassium leak and hypokalemia.
143
Q

significance of the translocation t(15;17) in AML?

A

confirms acute promyelocytic leukemia (APML; M3 subtype) and confers a good prognosis.

144
Q

What cytogenetic abnormality is associated with a better prognosis in the M2 subtype of AML?

A

translocation t(8;21).

145
Q

How does t(15;17) affect the morphological appearance of promyelocytes in APML?

A

Hypergranular promyelocytes are seen, except in the M3 variant, which is hypogranular.

146
Q

What is the utility of immunophenotyping in leukemia?

A

cell markers to distinguish AML from ALL if there are difficulties.

147
Q

What can be observed on a blood film showing AML?

A

blast cells

148
Q

Amyloidosis

A
  • abnormal protein, called amyloid, builds up in organs and tissues, impairing their function.
149
Q

Types of amyloidosis

A
  • AL (primary)
  • AA (secondary)
  • hereditary
  • familial amyloidosis
150
Q

AL amyloidosis proteins

A

light chains of immunoglobulins produced by plasma cells.

151
Q

conditions are commonly associated with AA amyloidosis?

A

chronic inflammatory conditions such as
* rheumatoid arthritis
* inflammatory bowel disease
* chronic infections.

152
Q

What protein accumulation characterizes hereditary amyloidosis?

A

accumulation of mutant transthyretin (TTR) protein.

153
Q

common symptoms of amyloidosis?

A
  • Fatigue
  • weight loss
  • swelling of the ankles and legs
  • shortness of breath
  • irregular heartbeats.
154
Q

How is amyloidosis diagnosed?

A
  • Biopsy of affected tissue
  • staining with Congo red dye
  • apple-green birefringence under polarized light.
  • further analysis with immunohistochemistry or mass spectrometry.
155
Q

Organs affected by amyloidosis

A
  • kidneys
  • heart
  • liver
  • nervous system
  • gastrointestinal tract.
156
Q

Vaccinations post Spelenctomy

A
  • Pneumococcal vaccination (with regular boosters every 5 years).
  • Seasonal influenza vaccination (yearly, typically every autumn).
  • Haemophilus influenza type B vaccination (one-off).
  • Meningitis C vaccination (one-off).

Low does phenoxymethylpenicillin or clarithromyocin or erythromycin for life

157
Q

Indications for splenectomy

A
  • trauma and rupture
  • hypersplenism
  • haemolytic anemia
158
Q

Non-Hodgkin’s lymphoma

A
  • Lymphoid malignancy
  • absence of Reed-sternberg cells
159
Q

Epidemiology of NHL

A
  • More common in males
  • affects all ages equally
160
Q

Infectious associations with NHL

A
  • Helicobacter pylori – gastric MALT (mucosa-associated lymphoma tissue) lymphoma
  • Epstein–Barr virus – Burkitt lymphoma (high-grade NHL) and AIDS-related CNS lymphoma
  • hepatitis C virus – diffuse large B-cell lymphoma and splenic marginal zone lymphoma
  • human T-cell lymphotropic virus type 1 (HTLV1) – T-cell lymphoma
  • HIV/AIDS
  • post-organ transplant
  • Autoimmunse disorder
  • inherited disorders affecting DNA repair
161
Q

Classification of NHL

A
  • 30 different types
  • cell of origin – B cell or T cell;
  • pathological grade – high grade (aggressive) or low grade (indolent)
162
Q

diffuse large B-cell lymphoma (DLBCL)

A
  • 60-70% cure rate and approaching 90% in young, fit patients
    *
163
Q

Prognosis of NHL

A

5-year survival for treated patients with:
Low-grade lymphoma is typically around 30%
High-grade disease is 50%

164
Q

NHL investigation

A
  • LDH blood test
  • Normocytic anaemia or haemolytic anaemia
  • Paraprotein
  • blood films
  • biospy
  • Cytogenetics (t(11;14)
165
Q

Low-grade, indolent NHL managment

A
  • asymptomatic patients ‘watch and wait’ until there is evidence of symptoms/organ failure
  • ** stage I disease**, local radiotherapy to nodal sites can be used
  • advanced systemic disease, immunochemotherapy is usually given – ‘rituximab in combination with chemotherapy or obinutuzumab with chemotherapy is now the preferred therapy for most indolent NHLs.
166
Q

Sickle cell anaemia

A
  • normal haemoglobin (where variable HbS is present) tends to form abnormal haemoglobin molecules
  • (HbS) upon deoxygenation leading to distortion of RBCs.
167
Q

Epidemiology of sickle cell disease

A

Central and West African descent

168
Q

Vaso-occlusive crises

A
  • Primarily caused by microvascular obstruction due to RBC sickling and inflammation
  • May be triggered by local hypoxia (eg. in cold weather)
169
Q

Vaso-occlusive crisis symptoms

A

severe pain
swelling
fever.
It can affect various parts of the body, such as the bones, joints, and abdomen.

170
Q

Managment of Vaso-occlusive crises

A
  • analgesics
  • hydration
  • oxygen therapy
  • treatment of any underlying infections or triggers.
171
Q

complications of SCD

A
  • Splenic infarction and subsequent immunocompromise
  • Sequestration crisis
  • Osteomyelitis
  • Stroke
  • Dactylitis
  • Poor growth
  • Chronic renal disease
  • Gallstones
  • Retinal disorders
  • Priapism
  • Pulmonary fibrosis and pulmonary hypertension
  • Iron overload from repeated blood transfusions
  • Red cell aplasia (due to parvovirus B19 infection in the presence of chronic haemolytic anaemia)
172
Q
A
173
Q

SCD managment

A
  • peak flow
  • FBC
  • Reticulocytosis and unconjugated hyperbilirubinemia may be noted
174
Q

SCD blood film finding

A
  • characteristic sickle cells
  • target cells
  • reticulocytosis with polychromasia
  • features of functional hyposplenism (Howell–Jolly bodies, nucleated red cells)
175
Q

SCD frequent crises managment

A

Hydroxycarbamide

176
Q

SCD newer treatment

A
  • crizaniluzumab (p-selectin inhibitor) for treatment of pain crises
  • voxelotor (haemoglobin oxygen-affinity modulator) for haemolytic complications

UKMLA Prep (38 decks)

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