Haematology and Oncology Flashcards

Question

What are the clinical features of hereditary spherocytosis?

Answer 1

May be asymptomatic Jaundice - in childhood, may be intermittent Anaemia Mild to mod splenomegaly Aplastic crisis - uncommon, transient (2-4 wks) caused by parvovirus B19 infection Gallstones - due to increased bilirubin excretion

Answer 2

Most are mild, need oral folic acid as have raised requirement due to increased RBC production. Splenectomy beneficial, indicated in poor growth or troublesome anaemia - tired, loss of vigour Need to ensure vaccinated against Hib, men C, strep pneumonia Lifelong daily oral penicillin prophylaxis

Answer 3

Duodenum and jejunum Requires acid from stomach to keep iron as soluble ferrous That is why meds e.g. PPIs can interfere with iron absorption, as less acid

Answer 4

The total space on transferrin molecules which iron as ferric irons Fe3+ can bind to the transferrin. This is measured as transferrin saturation - how much is bound.

Answer 5

Extra ferritin released if inflammation e.g. cancer, infection Normal or low - iron deficiency anaemia could be normal if they also have an infection

Answer 6

High in iron deficiency | Low in iron overload

Answer 7

Ferrous sulphate or ferrous fumarate Can cause constipation and black coloured stools Unsuitable if malabsorption is the cause

Answer 8

Condition where there is a defect in the G6PD enzyme X linked recessive Usually affects males

Answer 9

Infections Medications e.g. antimalarials Broad beans/fava beans Becomes jaundiced and anaemic after e.g. eating broad beans

Answer 10

Protects cells from damage by reactive oxygen species Deficiency makes RBCs more vulnerable to ROS, leading to haemolysis So periods of increased stress can lead to more ros, acute haemolytic anaemia

Answer 11

Neonatal jaundice Anaemia Intermittent jaundice Gallstones Splenomegaly Heinz bodies seen on blood film - denatured Hb Diagnosis can be made by doing a G6PD enzyme assay

Answer 12

Avoid triggers to acute haemolysis - including fava beans and medications Also napthalene in mothballs ``` Primaquine - antimalarial Ciprofloxacin Nitrofurantoin Trimethoprim Sulfonylureas Sulfasalazine ```

Answer 13

Pregnant women at risk of being carriers offered testing during pregnancy Newborn screening heel prick at 5 days of age

Answer 14

Autosomal recessive Abnormal gene on chromosome 11 Two copies needed for trait Abnormal HbS variant

Answer 15

Anaemia Increased risk of infection Avascular necrosis of large joints such as hip Pulmonary hypertension Painful and persistent erection - priapism CKD Sickle cell crises - vaso-occlusive crises; sickle cells clump together and block small blood vessels Acute chest syndrome

Answer 16

``` Managed supportively Have low threshold for admission to hospital Treat any infection Keep warm, IV fluids Simple analgesia Penile aspiration to treat priapism ```

Answer 17

Avoid dehydration and other triggers of crises, often have high haematocrit Ensure vaccines up to date Antibiotic prophylaxis e.g. penicillin V against infection Hydroxycarbamide can be used to stimulate HbF Blood transfusion in severe anaemia Bone marrow transplant can be curative

Answer 18

RBCs blocking blood flow in the spleen Causes acutely enlarged and painful spleen Pooling of blood in spleen can lead to severe anaemia and circulatory collapse Emergency Supportive management with blood transfusions and fluid resuscitation Splenectomy prevents this Used in recurrent crises

Answer 19

Temporary loss of creation of new blood cells Often due to infection with parvovirus B19 Leads to significant anaemia, management supportive with blood transfusions Resolves spontaneously within a week

Answer 20

Fever and resp symptoms, and new infiltrates on CXR Can be due to infection e.g. pneumonia or bronchiolitis, or non infective causes e.g. vaso-occlusion or fat emboli

Answer 21

Abx or antivirals for infection Blood transfusions for anaemia Incentive spirometry to encourage effective and deep breathing Artificial ventilation with NIV or intubation

Answer 22

Cancer of a particular line of stem cells in bone marrow Causes unregulated production of certain blood cells Classified by chronic (slow) or acute (fast) and cell line affected - myeloid or lymphoid

Answer 23

Most common in acute lymphoblastic leukaemia Acute myeloid leukaemia Chronic myeloid leukaemia is rare

Answer 24

Under 2 years

Answer 25

Mutation leads to excessive production of a type of white blood cell Overproduction of single type of cell can lead to suppression of other cell lines Causes underproduction of other cell types Results in pancytopenia Low RBCs - anaemia Low WBCs - leukopenia Low platelets - thrombocytopenia

Answer 26

Radiation exposure e.g. AXR in pregnancy, main environmental risk factor for leukaemia Down's Kleinfelter syndrome Noonan syndrome Fanconi's anaemia

Answer 27

``` Non-specific Persistent fatigue Unexplained fever Failure to thrive Weight loss Night sweats Pallor - anaemia Petechiae Abnormal bruising - thrombocytopenia Unexplained bleeding - thrombocytopenia Abdominal pain Generalised lymphadenopathy Unexplained or persistent bone or joint pain Hepatosplenomegaly ```

Answer 28

Any child with unexplained petechiae or hepatomegaly for immediate specialist assessment

Answer 29

FBC - anaemia, leukopenia, thrombocytopenia, high numbers of abnormal WBCs Blood film - blast cells Bone marrow biopsy Lymph node biopsy CXR CT scan Lumbar puncture Genetic analysis and immunophenotyping of abnormal cells

Answer 30

Chemo Radio Bone marrow transplant Surgery

Answer 31

``` Failure to treat Stunted growth and development Immunodeficiency and infections Neurotoxicity Infertility Secondary malignancy Cardiotoxicity ```

Answer 32

A localised mass The consequences of disseminated disease e.g. bone marrow infiltration - causes system ill health Consequences of pressure from a mass on local structures or tissue

Answer 33

Primary curative treatment e.g. acute lymphoblastic leukaemia To control primary or metastatic disease before definitive local treatment with surgery and/or radiotherapy As adjuvant treatment to deal with residual disease and to eliminate presumed micrometastases e.g. after initial local treatment with surgery in Wilms tumour

Answer 34

``` Bone marrow suppression Immunosuppression Gut mucosal damage Nausea and vomiting GI damage, painful mouth ulcers can prevent eating Anorexia Alopecia Drug specific side effects ```

Answer 35

Risk of serious infection | Children with fever and neutropenia admitted for cultures and broad spectrum antibiotics

Answer 36

Pneumocystis jiroveci pneumonia - especially in children with leukaemia Disseminated fungal infection - e.g. aspergillosis and candidiasis Coagulase negative staphylococcal infections of central venous catheters Measles and chickenpox may have an atypical presentation Use of live vaccines contraindicated during chemo, and 6 months after

Answer 37

Induction Consolidation and CNS protection e.g. methotrexate Interim maintenance e.g. monthly vincristine and dexamethasone Delayed intensification Continuing maintenance - continues for further 2 years in girls, 3 years in boys

Answer 38

Results from genetic alterations which trigger abnormal proliferation of lymphocytes

Answer 39

In lymphoma, the malignant cells are mature lymphocytes Arise outside of the bone marrow e.g. lymph nodes Leukaemia is from immature blasts in the bone marrow EXCEPT lymphoblastic lymphomas - B and T cell lymphoblastic lymphomas develop from precursors

Answer 40

Degree of bone marrow infiltration by blasts <25% involvement is lymphoma, >25% leukaemia

Answer 41

Hodgkin's: ``` Classical: Nodular sclerosis Mixed cellularity Lymphocyte rich Lymphocyte depleted ``` Non-classical: Nodular lymphocyte predominant HL (Reed sternberg cells not present) Non-Hodgkin's: Majority are high grade lymphomas (mature cell): - B cell e.g. Burkitt's lymphoma Large B cell lymphoma Primary CNS lymphomas - T cell Anaplastic large cell lymphoma Peripheral T cell lymphoma Mature cell: low grade: - B cell - follicular - T cell - rare Precursor: B and T cell lymphoblastic lymphoma

Answer 42

Immunodeficiency - post solid organ transplant, ataxia telangiectasia, HIV, immunosuppressive drugs

Answer 43

Painless progressive lymphadenopathy - develops over weeks/months (could just be due to infection, so requires careful history) B symptoms: fatigue, drenching night sweats, fever <38, weight loss >10% 6 mnths Pruritus Mediastinal involvement (thymus or mediastinal lymph nodes) dyspnoea, cough, chest pain

Answer 44

More common in NHL Bone marrow - symptoms of anaemia, infection, easy bruising and bleeding Abdomen - bloating, early satiety, pain, unable to pass stools and vomiting if obstructed Retroperitoneal lymphadenopathy - urinary retention Skin - new skin lesions, jaundice Testicular swelling CNS - behavioural change, headache, confusion, nausea and vomiting, seizures, weakness, sensory changes

Answer 45

Non tender, firm, matted lymph nodes In Hodgkin's - cervical, supraclavicular, axillary In NHL - more rapidly growing and bulky Mediastinal mass - may cause SVC obstruction, effusions, airway obstruction Abdomen - splenomegaly, hepatomegaly, abdominal mass Skin - T cell lymphomas inc mycosis fungoides, jaundice Testicular mass Neurological weakness, sensory abnormalities, features of raised ICP

Answer 46

Viral infective - URTI, mono, rubella, measles, CMV, adenovirus, HIV Bacterial infection - TB, typhoid, syphillis, lyme's, brucellosis Malignant - leukaemia, neuroblastoma Autoimmune - JIA, SLE, drug reaxtions

Answer 47

Bedside: Pbs, swabs (rule out infection) urine dip, ECG - check before starting cardiotoxic chemo Bloods: FBC - pancytopenia or leukaemia, (anaemia, thrombocytopenia, raised WBC in leukaemia) U&Es - baseline kidney function LDH and urate - raised in high cell turnover LFTs, low albumin = worse prognosis Monospot test - exclude EBV Hep B/HIV risk of reactivation with rituximab G6PD def identified as tx can cause haemolytic crisis ESR - worse prognosis if raised Imaging: CXR - mass CT/MRI/PET - staging USS liver or spleen Biopsies of enlarged node Fine needle aspiration Excision or partial biopsy

Answer 48

Ann Arbor for Hodgkin's St Jude's for non-Hodgkin's I - 1 group on 1 side of diaphragm II - >2 groups same side III - nodes on both side IV Hodgkin's - extranodal sites beyond E IV NHL - disseminated, multifocal A: no systemic symptoms B: systemic symptoms

Answer 49

Chemo Radio <50% children, PET scan assess response after 2 cycles of chemo and if still going, need adjuvant radiotherapy Lymphocyte predominant HL has less intensive tx - usually surgery or low dose chemo sufficient

Answer 50

Chemo Regime depends on type and staging B cell usually 4-6 invasive T cell NHL less invasive, lasts 2-3 years Biologics e.g. rituximab Radio as adjuvant rarely Bone marrow transplant for relapsed patients Lymphoblastic lymphoma treated according to ALL

Answer 51

``` SVC obstruction Bowel obstruction/perforation Cytopenias Pericardial or pleural effusions Pain from tumour invasion Tumour lysis syndrome ```

Answer 52

``` Neutropenic sepsis Mucositis, diarrhoea Anorexia, weight loss Alopecia Nausea and vomiting Fatigue Tumour lysis syndrome ```

Answer 53

``` Secondary cancers Cardiotoxicity Pulmonary toxicity Renal impairment Growth impairment Infertility ```

Answer 54

Oncological emergency caused by lysis of tumour cells either due to chemo or spontaneously in highly proliferative tumours

Answer 55

Hyperphosphataemia Hyperkalaemia Hypocalcaemia Hyperuricaemia AKI Cardiac arrhythmias Nausea and vomiting Seizures

Answer 56

Prophylactic hydration Allopurinol Prior to chemo Rasburicase can be used if white cell count >50, breaks down uric acid (check no G6PD def) Allopurinol prevents uric acid production

Answer 57

``` Aggressive hydration Rasburicase - drug mimics urate oxidase (not present in humans) Allopurinol Haemofiltration Dialysis ```

Answer 58

``` Disseminated intravascular coagulation Systemic activation of blood coagulation Generates intravascular fibrin Leads to thrombosis of small and medium sized vessels Organ dysfunction ```

Answer 59

Severe sepsis or shock due to circulatory collapse e.g. meningococcal sepsis damage from trauma or burns May be acute or chronic Likely initiated through tissue factor pathway

Answer 60

Bruising Purpura Haemorrhage Pathophysiological process - microvascular thrombosis, purpura fulminans

Answer 61

``` No single test Thrombocytopenia Prolonged prothrombin time Prolonged APTT Low fibrinogen Raised fibrinogen degradation products D-dimers Microangiopathic haemolytic anaemia ```

Answer 62

Treat underlying cause of DIC, usually sepsis Intensive care Provide fresh frozen plasma to replace clotting factors Cryoprecipitate Platelets

Answer 63

``` Spleen contains red pulp - filtration of RBCs white pulp - active in immune response creation of RBCS if not in bone marrow storage of RBCs, lymphocytes, platelets in emergency ``` production and maturation of B and T cells and plasma cells

Answer 64

Planned Traumatic - accident or surgery Autosplenectomy - physiological loss of spleen function (hyposplenism)

Answer 65

``` Sickle cell anaemia - chronic damage to spleen results in atrophy coeliac disease dermatitis herpetiformis essential thrombocythaemia ulcerative colitis ```

Answer 66

Trauma Spontaneous rupture - often in massive splenomegaly e.g. mono with a minor trauma Hypersplenism - hereditary spherocytosis, elliptocytosis, immune thrombocytopenia Neoplasia - lymphoma or leukaemia Cysts, abscesses

Answer 67

Howell-Jolly bodies Basophilic nuclear remnants in circulating erythrocytes During maturation, late erythrocytes usually expel nuclei, some do not but are filtered by the spleen Therefore indicates a damage or absent spleen

Answer 68

Individuals with absent or dysfunctional spleen at increased risk of severe infection - encapsulated bacteria S pneumonia; pneumococcus H influenzae Hib N meningitidis Increased risk of severe falciparum malaria

Answer 69

Immunisations against s pneumoniae, n meningitidis, Hib, influenza Antibiotic prophylaxis - oral phenoxymethylpenicillin or macrolides If unwell, systemic antibiotics and admit If at high risk in hyposplenism e.g.: age <16, or >50 years Poor response to pneumococcal vaccination Previous invasive pneumococcal illness Underlying haematological malignancy resulting in splenectomy

Answer 70

Low levels of red blood cells, white blood cells and platelets

Answer 71

Acute leukaemias Bone marrow failure Megaloblastic anaemia Can be due to reduced production: (Or increased removal - rare) B12/folate deficiency Drugs - chemo, abx, anticonvulsants, psychotropic drugs, DMARDs Viruses Malignancy Radiation Idiopathic aplastic anaemia Congenital bone marrow failure e.g. Fanconi's anaemia

Answer 72

``` Tired, weak, dizzy, SOB Frequent fevers, infection Pale skin, petechiae Bleeding Bruising easily Heavy menstruation Blood in bowel movements ```

Answer 73

Bloods, bone marrow biopsy/aspiration Blood transfusion Stem cell transplant

Answer 74

Antimalarials e.g. primaquine, quinine, chloroquine Abx Sulphonamides Quinolones e.g. ciprofloxacin Nitrofurantoin Aspirin in high doses Chemicals Naphthalene - mothballs Fava beans - divicine

Answer 75

Affected children inherit HbS from one parent, and HbC from another whereas sickle cell anaemia is HbSS and both HbAs replaced with HbS and HbC so no normal HbA - no normal beta globulin genes Fewer painful crises than those with HbSS But may develop proliferative retinopathy so have eyes checked

Answer 76

Beta thalassaemia major - HbA not produced Beta thalassaemia intermedia - small amount HbA produced, anaemia not bad enough to need blood transfusions Minor - one normal gene, heterozygous Small amount of HbA produced

Answer 77

Low level of intracellular haemoglobin leads to hypochromic microcytic cells

Answer 78

Silent carrier state Deletion of a single α-globin gene. It is asymptomatic, without anaemia α-Thalassemia trait Deletion of two α-globin genes There is minimal or no anaemia and no physical signs; findings are identical to those of β-thalassemia minor (microcytosis and hypochromia) Haemoglobin H (HbH) disease Deletion of three α-globin genes. Tetramers of β-globin, called HbH, are formed There is moderately severe anaemia, resembling β-thalassemia intermedia (microcytic, hypochromic anaemia with target cells and Heinz bodies in the blood film) Hydrops fetalis Deletion of all four α-globin genes In the foetus, excess of γ-globin chains form tetramers (Hb Bart) that are unable to deliver the oxygen to tissues. Usually intrauterine death

Answer 79

Severe anaemia, transfusion dependent Failure to thrive Extramedullary haemopoesis - prevented by regular transfusions ``` Pallor Jaundice Bossing of skull Maxillary overgrowth Splenomegaly Hepatomegaly Need for repeated transfusions ```

Answer 80

Iron deposition - cardiomyopathy, cirrhosis, diabetes, delayed growth, hyperpigmentation Alloantibodies makes finding compatible blood hard Infection - rare HIV, Hep A B C, prions Venous access difficult, device may be needed Could then cause infection

Answer 81

Inherited form of aplastic anaemia Congenital abnormalities - short stature, abnormal thumbs, renal malformations At high risk of death from bone marrow failure or transformation to acute leukaemia

Answer 82

Pancytopenia | Hypocellular bone marrow in absence of abnormal infiltrate or marrow fibrosis

Answer 83

X linked recessive

Answer 84

Spontaneous bleeding into joints and muscles Can lead to crippling arthritis In neonatal period, risk of intracranial haemorrhage Haem A - prophylactic FVIII Haem B - FIX

Answer 85

Autosomal dominant Carriers can still have some deficiency Defective platelet plug Bruising Excessive prolonged bleeding after surgery Mucosal bleeding e.g. epistaxis, menorrhagia

Answer 86

Haemorrhagic disease of newborn due to Vit K deficiency Liver disease Immune thrombocytopenia DIC

Answer 87

Inadequate intake Malabsorption - coeliac, CF, obstructive jaundice Vit K antagonists e.g. warfarin

Answer 88

Idiopathic thrombocytopenic purpura Characterised by idiopathic thrombocytopenia (low platelet count) causing a purpuric non-blanching rash Type II hypersensitivity reaction Produces antibodies which destroy platelets

Answer 89

Usually <10s Hx recent viral illness Onset of symptoms over 24-48 hrs Bleeding - gums, epistaxis, menorrhagia Bruising Petechial, purpuric rash - bleeding under skin

Answer 90

Large area of blood collected under the skin more than 10 mm | Non bllanching lesion

Answer 91

FBC, platelet count Exclude other causes of low platelet count - e.g. heparin induced, or leukaemia Tx only needed if severe thrombocytopenia - below 10, or patient actively bleeding Prednisolone IV immunoglobulins Blood transfusions Platelet transfusions work temporarily as antibodies will attack again

Answer 92

Avoid contact sports Avoid IM injections, procedures such as LPs Avoid NSAIDs, blood thinners Advice on managing nosebleeds Seek help after any injury that could have caused internal bleeding

Answer 93

Chronic ITP Anaemia Intracranial and subarachnoid haemorrhage GI bleeding