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Phase 2A conditions > Haematology Conditions > Flashcards

Haematology Conditions Flashcards

1
Q

What can cause Iron deficiency anaemia?

A

blood loss (menorrhagia or GI bleeding), Poor diet, increased demands (pregnancy) or malabsorption

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2
Q

What are RF for iron deficiency anaemia?

A

females, underdeveloped country, pregnancy, premature infants

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3
Q

What symptoms would you expect in iron deficiency anaemia?

A

fatigue, headaches, palpitations, pallor, brittle hair and nails, spoon-shaped nails, angular stomatitis

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4
Q

What lab results do you order and what results do you expect in iron deficient anaemia?

A

Order FBC and film (microcytic and hypochromic, poikilocytosis), serum iron (total iron binding capacity rises), serum ferritin, serum soluble transferrin receptors (increased)

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5
Q

How do you treat iron deficiency anaemia?

A

Treat the cause, ferrous sulphate or if SE bad give ferrous gluconate
- extreme cases give IV iron

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6
Q

What are causes of folate deficiency?

A

poor dietary intake, increased demand, malabsorption or antifolate drugs (methotextrate)

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7
Q

What are the RF for folate deficiency?

A

elderly, alcoholic, pregnant, Crohn’s or coeliac

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8
Q

How does folate deficiency present?

A

Can be asymptomatic or present with general anaemia symptoms, glossitis (sore red tongue)
no neurological involvement (distinguish from B12)

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9
Q

What tests might you order and what results do expect in folate deficiency?

A

FBC and film (macrocytic and megaloblastic), low serum and red cell folate, serum bilirubin may be raised

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10
Q

How do you treat folate deficiency?

A

Treat underlying cause and give folic acid tablets

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11
Q

What causes sickle cell anaemia?

A

Genetic defect results in sickle cell Hb (Hbs) which causes anaemia
Recessive single gene mutation replaces glutamic acid with valine in the beta-globin chain

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12
Q

What is the difference between sickle cell anaemia and sickle cell trait?

A

If only one genetic copy is affected it is called sickle cell trait

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13
Q

What is the pathophysiology of sickle cell?

A

Triggering factors (hypoxia, infection etc) can cause polymerisation of Hbs, leading cells to become ridged and deformed: occlusion and adherence to endothelium, decreased oxygen affinity

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14
Q

How does sickle cell present and what lab results would you expect?

A

Presents with skeletal pain, chest pain, dactylits (swollen hands), features of anaemia and failure to thrive

elevated reticulocyte count, visual change on blood film, cloudy solubility test, electrophoresis shows Hbs (not HbA)

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15
Q

What tests would you order to test for sickle cell?

A

DNA assay, FBC and film, Hb electrophoresis, solubility test

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16
Q

What complications can sickle cell have?

A

anaemia, chronic pain, cardiac failure, gallstones

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17
Q

How do you treat sickle cell disease?

A

Prevention of crisis is key: avoid dehydration, coldness, exhaustion and smoking

Hydroxyurea (decreases adhesion), L-glutamine (reduces sickling and analgesic
Blood transfusions

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18
Q

What are two types of haemolytic anaemia

A

Extravascular destruction: immune targeting by antibiotics

Intravascular: lysis or direct trauma

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19
Q

What is haemolytic anaemia?

A

Umbrella term for conditions that result in premature destruction of RBC

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20
Q

what causes haemolytic anaemia?

A

hereditary (sickle cell or enzyme deficiencies) or acquired (autoantibodies, drugs, infections, pregnancy)

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21
Q

How does haemolytic anaemia present?

A

pallor, fatigue, jaundice, shortness of breath

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22
Q

What tests would you order to test for haemolytic anaemia?

A

FBC and smear, MCHC, reticulocyte count, bilirubin, Coombs test for autoantibodies

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23
Q

what lab results would you expect in haemolytic anaemia?

A

ow Hb, increased MCHC (Hb concentration), elevated bilirubin, increased reticulocyte percentage, coombs can be positive or negative

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24
Q

How do you treat haemolytic anaemia?

A 
Inherited cause: folic acid
Coombs positive (autoimmune): folic acid, corticosteroids
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25
What causes B12 deficiency?
dietary, malabsorption and pernicious anaemia
26
what is pernicious anaemia?
B12 deficiency with an autoimmune cause: parietal cells are attacked causing loss of intrinsic factor
27
what are RF for B12 deficiency?
elderly, female, metformin use, pregnancy, northern European, chronic GI disease
28
How does B12 deficiency present?
``` Insidious onset (storage can last up to 4 years): general anaemia symptoms, light yellow skin (pallor and light jaundice), red sore tongue, angular stomatitis very low levels can cause neurological signs such as progressive weakness, symmetrical paresthesia, hallucinations or psychiatric problems ```
29
What do you have to differentiate B12 deficiency from?
Folate deficiency as folic acid can contribute to deficiency
30
What labs do you order and what results do you expect in B12 deficiency?
FBC and film (macrocytic and megaloblastic, oval macrocytes, neutrophil polymorphs), serum bilirubin, serum B12, intrinsic factor antibodies
31
How do you treat B12 deficiency?
``` Treat cause (if not pernicious): if dietary give oral B12, if malabsorptive give injections replenish stores with IM hydroxocobalamin ```
32
What causes DVT?
change to Virchow’s triad i.e. surgery, prolonged immobility, medications
33
what is Virchow's triad?
Factors important in the development of veinous thrombosis: change of blood flow (stasis) change of blood constituents and change to endothelium (damage)
34
What are FR for a DVT?
bedridden, major surgery in last 12 wks, active cancer, age, pregnancy, Protein C or S deficiency, obesity, smoking
35
How does a DVT present?
calf swelling, localised pain and prominent superficial veins (can progress to PE before presenting)
36
What do you use to assess likelihood of having a DVT or PE?
well's score: above 2 means likely
37
What tests do you order in a DVT?
venous ultrasound and D-dimer, LFT, FBC, clotting screen
38
What are differentials of DVT?
cellulitis and calf muscle tear
39
What lab results do you expect in a DVT?
D-dimer elevated, abnormal doppler ultrasound and abnormal B-mode image (lumen of vein cannot be compressed)
40
what is the order of events if well's score indicates likely?
1. ultrasound (if you can do within 4 hours) | 2 otherwise do d-dimer and then anticoagulation - try and do an ultrasound within 24 hours)
41
what is the order of events if well's score indicates unlikely?
1. D-dimer (within 4 hours) 2. If it takes longer than 4 hours give anticoagulation while waiting 3. if positive do ultrasound 4. if negative stop anticoagulant
42
How do you treat a DVT?
LMWH for 5 days followed by edoxaban
43
What is acute lymphoblastic leukaemia?
Monoclonal proliferation of lymphoid stem cells (lymphoblasts) in bone marrow (can be B or T)
44
What are RF for ALL?
young children, down syndrome, radiation exposure
45
How does ALL present?
abrupt onset: anaemia, thrombocytopenia, bone pain, lymphadenopathy, fatigue, hepatosplenomegaly
46
What tests would you order for ALL?
FCB and smear, bone marrow aspiration and Lumbar puncture
47
What results would you expect in ALL?
increased WCC, presence of lymphoblasts, if there is more than 20% of lymphoblasts in bone marrow it confirms the diagnosis
48
How do you treat ALL?
Treat with aggressive chemo (dexamethasone+ vincristine+ daunorubucin+ cyclophosphomate) Good prognosis in children
49
What is chronic lymphocytic leukaemia?
Monoclonal proliferation of mature, abnormal B cells in the bone marrow
50
What are causes of CLL?
chromosomal abnormalities and mutation of proteins
51
What are RF for CLL?
adult age, family history, agent orange exposure
52
How does CLL present?
late onset/starts asymptomatic: lymphadenopathy, hepatosplenomegaly, rubbery lymph nodes
53
What tests would you order for CLL?
FBC and smear, lymph node biopsy
54
What results do you expect in CLL?
anaemia, thrombocytopenia, presence of smudge cells on smear and raised WCC
55
What are complications of CLL?
abnormal Ig secretion and richter syndrome (procession to aggressive lymphoma)
56
How do you treat CLL?
Incurable: chemo and immunotherapy, bone marrow transplant
57
what is acute myeloid leukaemia?
Monoclonal proliferation of myelogenous stem cells in bone marrow
58
What are RF for AML?
older adults, radiation exposure, previous chemo, down syndrome, myeloproliferative disorders
59
How does AML present?
Abrupt onset: pallor, ecchymosis, anaemia, bone pain, gum swelling
60
What tests would you order for AML?
FBC and smear, bone marrow aspiration
61
What results would you expect in AML?
reduced number of RBC, auger rods, over 20% myoblasts in marrow
62
What complication can come with AML?
disseminated intravascular coagulation (DIC): development of small clots
63
How do you treat AML?
chemotherapy in intervals to allow for bone marrow recovery, transplant
64
What is chronic myeloid leukaemia?
Monoclonal proliferation of mature granulocytes
65
What are causes of CML?
Philadelphia chromosome (22) that creates fusion proteins and exposure to radiation
66
What are RF of CML?
60 to 75 years, radiation exposure
67
How does CML present and what are it's three phases?
Insidious onset and then rapid deterioration 1. chronic: asymptomatic, high WCC 2. accelerated: over 20% of basophils in marrow, just under 20 % of myeloblasts in marrow, anaemia, recurrent infections, ecchymoses 3. blast crisis: terminal phase with low survival rate, over 20% myeloblasts in marrow, bone pain, significant splenomegaly
68
What tests would you order for CML?
FBC and smear, bone marrow biopsy
69
How do you treat CML?
treat depending on phase: 1. chronic and accelerated: Tyrosine kinase inhibitor (imatinib) 2. blast crisis: TKI and high dose chemo, marrow transplant
70
What is hodgkin's lymphoma?
B or T cell malignancy in lymph nodes characterised by Reed-Sternberg cells (large, two nuclei, resembling owl eyes) RSC secrete cytokines which results in accumulation of inflammatory cells causing local and systemic effects
71
What are the different types of hodgkin's lymphoma?
Classic: different types with RSC | Nodular lymphocyte predominant: variation of Reed-Sternberg cells, highly curable, slow growing
72
How does HL present?
Presents with lymphadenopathy + B symptoms (additionally to lymph nodes, amount depends on subtype): fever, night sweats, weight loss
73
What tests would you order for HL and what results would you expect?
FBC, inflammatory markers, CXR, lymph node biopsy ESR elevated, potential raised WCC, mediastinal mass, presence of Reed-Sternberg cells
74
How do you treat HL?
Treat with ABVD regiment: doxorubicin, bleomycin, vinblastine and dacarbazine
75
What are the stages of HL?
- I: single node group - II: 2 or more regions, sane side of diaphragm - III: both sides of diaphragm - IV: extra lymphatic organ involvement
76
What is non hodgkin's lymphoma?
Group of malignancies of the lymphoid system without Reed-Sternberg cells 1. B-cell: different subtypes such as diffuse large B cell lymphoma (aggressive) or Burkitt (adolescents, very aggressive, starry sky appearance) 2. t-cell: adult T cell lymphoma (leukaemia)
77
How does NHL present?
painless lymphadenopathy, B symptoms, bowel obstruction, easy bruising, recurrent infections
78
How do you treat NHL?
Rituximab (monoclonal antibody that targets CD20)
79
What tests do you order for NHL?
CT and PET scan and do a lymph node biopsy
80
What is multiple myeloma?
Neoplasm of plasma cells in bone barrow due to overproduction of M protein myeloma cells secrete cytokines that promote proliferation which ensures survival of myeloma
81
What are the three types of MM?
- Smouldering MM (asymptomatic) - symptomatic MM - non-secretory MM
82
What are RF for MM?
alcohol consumption, obesity, radiation exposure and family history
83
How does MM present?
CRAB: calcium elevated, renal disease, anaemia, bone lesions
84
What tests would you order for MM and what results would you expect?
Imaging (X-ray, CT and MRI), FBC, electrolytes and marrow biopsy Bence Jones proteins, proteinuria, over 30% plasma cells in bone marrow, osteolytic lesions
85
How do you treat MM?
Treatable but incurable: chemotherapy (bortezomib, lenalidomide, dexamethasone), biphosphonates for bone loss stem cell transplant
86
What is malaria and its pathology?
Parasite infection (protozoa plasmodium) transmitted through mosquitos 1. infected mosquito injects sporozoites - travel to hepatocytes. 2. Reproduction occurs and after some time 30-40 thousand of matured versions enter the blood stream and RBC's . 3. They undergo further reproduction releasing more merozoites. 4. RBC rupture, release toxins and cytokines are released (symptoms)
87
What is the aetiology of malaria?
infected blood: P. falciparum
88
What are the RF of malaria?
travelled to tropical areas, no bed nets used, no prophylaxis, pregnancy, immunocompromised, extremities of ages
89
How does malaria present?
fever or a history of fever, headache, weakness and cough | severe forms include seizures and loss of conciousness
90
What tests do you order for malaria and what results do you expect?
1. FBC and smear with giemsa stain: shows parasite 2. rapid diagnostic test: good for p.falciparum but not for others 3. PCR blood
91
What are differentials of malaria?
yellow fever or zika virus
92
What are complications of malaria?
kidney injury, severe anaemia, organ failure and death
93
How do you treat malaria?
chloroquine unless p.falciparum (that's resistant). Use artemether or lumefantrine pregnant: quinine sulphate and clindamycin
94
How does Exoxaban work?
Factor Xa inhibitor: prevents conversion to thrombin and prolongs clotting time (reduces risk of thrombus formation)
95
How does Heparin work?
Anticoagulant: binds reversibly to antithrombin 3 which inactivates factors Xa and II and therefore prevents progression of existing clot
96
What is polycythemia vera?
ncreased blood cell levels (particularly RBC) due to overproduction by bone marrow
97
What causes polycythemia vera?
Mutation of JAK2 gene: always activated causing rapid proliferation
98
How does polycythemia vera present?
fatigue, dizziness, increased sweating, red face, blurred vision and itching skin
99
What results would you expect for polycythemia vera?
increased Hb, increased WCC and platelets, signs of fibrosis in marrow
100
What complications are associated with polycythemia vera?
DVT, stroke etc (due to being more prone to clots) and myelofibrosis (spent phase: all blood cells are low because there is scarring in marrow)
101
How do you treat polycythemia vera?
phlebotomy, myelosupressive medication (ruxolitnib a JAK2 inhibitor) blood transfusions in spent phase
102
what are the two types of haemophilia?
- A: factor 8 deficiency | - B: factor 9 deficiency
103
What are the symptom levels of haemophilia?
- mild (above 5): bleeding only with trauma and surgery - moderate: severe bleeding following injury and occasional spontaneous episodes - Severe (below 1): frequent spontaneous bleeding into muscles and joints
104
What tests do you order in haemophilia and what results do you expect?
Order PTT (normal) and APTT (prolonged as factor 8 and 9 are in intrinsic pathway), reduced plasma factor
105
How do you treat haemophilia?
IV recombinant factor concentrate, synthetic vasopressin
106
what is thrombocytopenia?
Deficiency of platelets in blood
107
What are the two types of thrombocytopenia?
1. Immune thrombocytopenia pupura (more common): autoimmune destruction 2. Thrombotic thrombocytopenia purpura: extensive clots so all the platelets are used up
108
What are causes of thrombocytopenia?
reduced platelet production, excess destruction, problems with an enlarged spleen
109
How does thrombocytopenia present?
easy bruising, purpura and fever
110
How do you treat thrombocytopenia?
1. immune: corticosteroids | 2. thrombotic: plasma exchange to remove antibodies, IV methylprednisolone, immunosuppression
111
what is thalassemia?
Blood disorder: decreased or no production of one or more global chains leading to reduced production and premature destruction of RBC
112
What are the two types of thalassemia?
- Alpha: decreased alpha chain synthesis | - Beta: decreased beta chain synthesis
113
What causes beta thalassemia?
genetic defects
114
What types of haemoglobin are present in beta thalassaemia?
excess alpha chains so they combine with whatever is available: HbA2 (alpha and delta) or HbF (alpha and gamma)
115
What is the symptom classification of beta thalassemia?
1. Minor (heterozygous): asymptomatic, mild anaemia, iron normal 2. intermedia: moderate anaemia, splenomegaly, gallstones, bone abnormalities 3. major (homozygous): presents in 1st year of life with severe anaemia, bony abnormalities, hepatosplenomegaly
116
How do you treat thalassemia?
transfusions (risk of iron overload), LT folic acid supplements
117
What tests do you order for thalassemia and what do you expect?
FBC and film (hypo chromic and microcytic, visible target cells), reticulocyte count decreased, skill xray (enlarged maxilla)
118
How do you diagnose thalassemia?
diagnose through Hb electrophoresis
119
what is von willebrandt disease?
Deficiency of van Willebrandt factor leading to less coagulation
120
How do you treat vWD?
tranexamic acid and desmopressin
121
How do you score the Well's score for DVT?
One point for each: active cancer, immobilisation of legs, bedridden for 3 days or surgery within 12 weeks, calf tenderness, swollen leg, swollen calf, pitting oedema on one leg, previous DVT likely: 2+

Phase 2A conditions (7 decks)

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