Pathology 5

Question 1

Types of B cell non Hodgkin lymphoma

Answer 1

Lymphoblastic B cell lymphomas,

Bcell: mature and high grade=
Diffuse large B cell lymphoma, mediastinal large B cell, primary CNS lymphoma, primary effusion lymphoma, Burkitts and Mantle lymphomas

Mature low grade=

Follicular lymphoma, MALT lymphoma, walaenstroms macroglobulinemia

Question 2

Types of T cell non Hodgkin lymphomas

Answer 2

Lymphoblastic T cell lymphomas,

Mature and high grade=
Enteropathy lymphoma, peripheral T cell lymphoma, subcutaneous anaplastic lymphoma, angioimmunoblastic lymphoma

Mature and low grade =
Mycosis fungoides, cutaneous T cell lymphoma

Question 3

Types of Hodgkin lymphomas

Answer 3

Classical HL= Nodular sclerosis type, lymphocyte rich, mixed cellularity, lymphocyte depleted. (Hodgkin / Reed sternberg cells)

Nodular lymphocyte predominant HL (shows popcorn cells)

Question 4

Classic Hodgkin lymphoma

Answer 4

Detected between 20-40 and again after 50-60. Signs include lymphadenopathy with cervical and axillary involvement. Nodes are enlarged, rubbery, discrete and mobile. Mediastinal involvement and masses in over half. Cough. Fever. Weight loss, night sweats. Can be respiratory. Predictable spread. Stages 1-4. Can get splenomegaly, bone and liver issues with it. Bad if anaemia and lymhocytopenic.
Can also have spleno/hepatomegaly, svc syndrome, cerebellum degeneration neuropathy or guillain barre.

Be sure these patients have their vaccinations
Barely breaches lymph node capsule. Appearance is Nodular and sclerotic. Oversized cells= Hodgkin cells, oversized and multinucleated= Reed sternberg cells. When both are present, referred to as HRS cells. Associated with the high grade primary mediastinal large B cell NHL.

No difference in prognosis and management of the 4 classes of classical HL. The Nodular type is most common

Question 5

Haemophilia

Answer 5

Excessive bleeding via Haemophilia A, B, C or Von willer and diseases. Haemophilia A is classic and B is Christmas disease.

Easy bruising, inc risk of bleeding within joints, compartments or the brain. In the brain shows as headaches, seizures, decreased consciousness.

Symptoms include internal or external bleeding, haematomas, swelling, deep muscle bleeding giving numbness or pain of limb.

Possible joint damage from haemarthritis making severe pain, disfigurement and arthritis.

Shows prolonged APTT in routine clotting screen. Diagnosis confirmed by factor assay. PT and platelets normal, but if recent bleed= low HB and haematocrit.

Question 6

Haemophilia A

Answer 6

Affects 1/4500 males as X linked recessive disorder. Becomes visible when a child begins to crawl. Lack of clotting factor 8. Characterised by a prominent tendency to haemorrhage. Failed intrinsic pathway. Moderate disease is 2-10 units/dl.

Question 7

Haemophilia B

Answer 7

Deficiency of factor 9, x linked inheritance. History and clinical presentation is milder. Many patients are asymptomatic until stressed by surgery or trauma.

Question 8

Haemophilia treatment

Answer 8

Factor replacement therapy - concentrates of 8 or 9 are slowly dropped or injected into a vein.

Question 9

Von willebrand disease

Answer 9

Group of haemorrhaging disorders in which the Von willebrand factor is abnormal. Hard to diagnose. They are usually inherited as autosomal dominant. Symptoms include prolonged bleeding time, deficiency of factor 8 and impaired platelet adhesion.

Question 10

Disseminated intravascular coagulation

Answer 10

Condition where small blood clots develop throughout the bloodstream, blocking small blood vessels. Clotting and bleeding everywhere.

The increased clotting depletes the platelets and clotting factors required to control bleeding. Appears like haemophilia.

Bleeding, bruising, low blood pressure, shortness of breath and confusion. It is acute and can lead to severe internal bleeding or organ failure. Caused by another underlying pathology eg infection or injury over activating the clotting system.

Question 11

Haemophilia C

Answer 11

Autosomal genetic disorder with a lack of clotting factor 11.

Question 12

Parahaemophillia

Answer 12

Mild factor 5 deficiency

Question 13

When can haemophilia A be acquired ?

Answer 13

Developing autoantibodies after/during cancer, autoimmune disorders and after childbirth.

Question 14

Use of Desmopressin

Answer 14

For mild haemophilia A, diabetes insipidus, VWF disease. It’s a synthetic ADH.

Question 15

Factor 5/ Leiden thrombophillia

Answer 15

Causes resistance to activated protein C. This means protein S can’t regulate factor 5, due to poor response to C. Factor 5 is slowly degraded. It’s prolongation enhances thrombinogen. Ten times risk of thrombosis as DVT, miscarriages etc.

Question 16

Prothrombin mutation thrombophilia

Answer 16

Triples the risk of thromboembolism. Causes elevated plasma prothrombin levels, possibly due to increased pre mRNA stability means prothrombin isn’t degraded and it accumulates. Factor 2 mutation.

Question 17

Protein c + s deficiency thrombophilia

Answer 17

Autosomal dominant. But an acquired deficiency can be result of liver disease, DIC, vitamin K deficiency or warfarin.

Symptoms = pain and tenderness where the clot is, skin necrosis, redness or swelling, treated with blood thinners eg warfarin.

Complications include childhood stroke, recurrent miscarriage, recurrent DVT, PE.

Question 18

Antithrombin deficiency thrombophilia

Answer 18

Can be congenital or acquired. Increases risk of venous thrombosis. Also affects other serine proteases of the coagulation cascade. Serpin 1 mutation. Gives a higher risk of DVT and risk increases past 55.

Tests for antithrombin deficiency = PTT, coagulation tests, gene tests.

To manage risk for VTE: warfarin or morevan anticoagulant. Inhibits vitamin k epoxide reductase, an enzyme that recycles oxidised vitamin K. Warfarin inhibits production of protein c + s so may temporarily make clotting worse, precipitate clots or necrosis. Give heparin before.

Question 19

Thrombolytic therapy for thrombophilia

Answer 19

Thrombolytic agents dissolve fresh clots and restore latency faster than anticoagulants. By injecting clot dissolving drugs into a patient’s veins.

Streptokinase, urokinase, tissue plasminogen activator works via activating fibrinolytic pathways. Usually a catheter is inserted, eg via femoral artery access to deliver to the clot directly.

Question 20

Pain relief options in children

Answer 20

Acute: paracetamol, ibuprofen, entonox, oromorph, lidocaine/EMLA cream

Chronic: amitriptyline, gabapentin, oromorph, paracetamol, ibuprofen

Intrathecal baclofen for reducing spasticity pain with cerebral palsy
Pizotifen for recurrent abdominal pain
Famotidine for dyspepsia
Peppermint oil for ibs 
No codeine for under 12s

Question 21

Complex regional pain syndrome/ reflex sympathetic dystrophy

Answer 21

Continuing or recurrent spontaneous/evoked pain disproportionate to the cause by time or extremity. Can manifest as extreme pain, swelling, limited range of motion, changes to skin (want, shiny,thin, tight) and bone thinning. Burning, stabbing, grinding and throbbing. Could be allodynia- pain to non painful stimuli. Can have autonomic signs too like local temperature change, cyanosis, oedema.

Associated with injury, surgery, heart attack, stroke, hemiplegia, smoking, RSD, stress, anxiety or depression.

Treatment can be supportive home based things, or transcutaneous electrical nerve stimulation, nerve block with biphosphonates, calcitonin, ketamine, botulinum, opioids

Question 22

Streptococcal Infections

Answer 22

Different groups of these bacteria are spread in different ways—for example, through coughing or sneezing, through contact with infected wounds or sores, or during vaginal delivery (from mother to child).
These infections affect various areas of the body, including the throat, middle ear, sinuses, lungs, skin, tissue under the skin, heart valves, and bloodstream.
Symptoms may include red and painful swollen tissues, scabby sores, sore (strep) throat, and a rash, depending on the area affected. Sinusitis, mastoiditis, tonsillitis.

Question 23

Groups of Streptococcal Infections

Answer 23

Group A, B, D (enterococcus), viridans

Question 24

Streptococcus A

Answer 24

Streptococcus pyogenes: gives otitis media, sinusitis, pharyngitis/strep throat, cellulitis,erysipelas, impetigo, endocarditis, necrotising fasciitis, pleurisy, pneumonia, scarlet fever, streptococcal toxic shock syndrome.

Disorders that develop after: glomerulonephritis and rheumatic fever

Question 25

Streptococcus B

Answer 25

Streptococcus agalactiae can cause abscess, cellulitis, diabetic foot infections. Systemic infection in newborns, meningitis and pneumonia, post delivery endometritis.

Question 26

Streptococcus viridans

Answer 26

Causes systemic infection, endometritis, caries, meningitis, endocarditis

Question 27

Strep throat

Answer 27

Fever Enlarged and tender lymph nodes in the neck Pus in or on the tonsils Absence of cough The main reason for diagnosing strep throat is to reduce the chance of developing complications (such as rheumatic fever) by using antibiotics.

Question 28

Signs of Strep B in newborns

Answer 28

Newborn babies with group B strep usually have signs in the first 24 hours after birth. These signs may include: Being fussy, very sleepy, and having breathing problems (signs of sepsis) Breathing fast and making grunting noises (signs of pneumonia) Having breathing problems and periods of not breathing (signs of meningitis) Having a change in blood pressure Having convulsions (seizure) Babies who get group B strep a week or so after birth may have signs such as: Decreased movement of an arm or leg Pain with movement of an arm or leg Breathing problems Fever Red area on the face or other part of the body Pregnant women may have group B strep without symptoms. When they have symptoms, these may include: ``` Having to urinate often, having an urge to go, or pain when urinating Fever Nausea and vomiting Pain in your side or back Uterus or belly is sore Fast heart rate ```

Question 29

Kawasaki disease/infantile polyarteritis nodosa

Answer 29

Kawasaki disease is an idiopathic self-limiting systemic vasculitis that most often affects children in the age range 6 months to 5 years. Chinese and Japanese more likely. Has a major complication of coronary artery aneurysm formation. It has taken over from rheumatic fever as the most common cause of acquired childhood heart disease. Fever lasting ≥5 days. Marked irritability of the child. Erythema, swelling and desquamation (from 2 weeks) affecting the skin of the extremities. Bilateral dry conjunctivitis. Wide spread non vesicular rash within 3-5 days starts on palms, soles and perineum. Inflammation of the lips, mouth, hands or feet and/or tongue. Cervical lymphadenopathy Possibly diarrhoea, vomiting, abdominal pain, urethritis. Cardiovascular signs are usually nonspecific. Tachycardia, a hyperdynamic precordium, a gallop rhythm or a flow murmur may be present; however, these signs are not unusual in febrile patients without Kawasaki disease. There are occasionally signs of valvular incompetence. Other possible signs include: Neck stiffness due to aseptic meningitis. Hepatomegaly and jaundice.

Question 30

Kawasaki treatment

Answer 30

The use of aspirin and intravenous immunoglobulin (IVIg) to reduce fever and myocardial inflammation and to prevent or ameliorate cardiac sequelae. Prednisone can be added. In other diseases that are febrile don’t use aspirin due to risk of Reye’s syndrome.

Question 31

Rheumatic fever

Answer 31

Rheumatic fever (acute rheumatic fever) is a disease that can affect the heart, joints, brain, and skin. Rheumatic fever can develop if strep throat and scarlet fever infections are not treated properly 2-4 weeks after the illness appears. Often after group A strep. The most common symptoms are: nodules, sore throat; swollen, red tonsils; fever; headache; and joint and muscle aches, especially in the knees. Risk endocarditis, myocarditis and pericarditis. Signs of these can be cardiomegaly,congestive heart failure, and pericardial friction rubs or leakage of blood from vessels. If the disorder affects the nervous system the patient may present with abrupt, non-repetitive limb movements and grimaces (Sydenham’s chorea). Treat with corticosteroids and penicillin, sulfadiazine, or erythromycin, are taken over a period of time to prevent recurrence.

Question 32

Bronchiolitis

Answer 32

bronchiolitis should be considered in children under the age of 2 years who present with a 1- to 3-day history of coryzal symptoms, followed by: Persistent cough; and Either tachypnoea or chest recession (or both); and Either wheeze or crackles on chest auscultation (or both). Other typical features include fever (usually of less than 39°C) and poor feeding. Consider an alternative diagnosis such as pneumonia if temperature is higher and crackles are focal. Consider viral-induced wheeze if there is wheeze without crackles, episodic symptoms and/or a family history of atopy. Marked chest recession or grunting. Respiratory rate >70 breaths/minute. Central cyanosis. Oxygen saturation of less than 92% Very young babies may present with apnoea alone.

Question 33

Entonox

Answer 33

Entonox is a gas which you breathe in to help make pain better. It can also produce feelings of relaxation, which can help you to feel less nervous about being treated by the doctors and nurses. It is a mixture of 50% nitrous oxide and 50% oxygen. Drowsy, dizziness, nausea and a dry mouth.

Question 34

Motor neurone disease

Answer 34

This is a degenerative condition that affects motor neurons, namely the anterior horn cells of the spinal cord and the motor cranial nuclei. It causes lower motor neurone (LMN) and upper motor neurone (UMN) dysfunction, leading to a mixed UMN/LMN picture of muscular paralysis, usually with LMN signs predominating. Most cases of MND are due to amyotrophic lateral sclerosis (ALS). However, other forms occasionally occur. These include: Progressive bulbar palsy - the muscles first affected are those used for talking, chewing and swallowing, Progressive muscular atrophy - this is an uncommon form of MND. The small muscles of the hands and feet are usually first affected, but muscle spasticity is absent, Primary lateral sclerosis - this is a rare type of MND. It mainly causes weakness in the leg muscles. Some patients with this type may also develop clumsiness in the hands or develop speech problems.

Question 35

Motor neurone disease symptoms

Answer 35

These symptoms may include[1]: Functional effects of muscle weakness, such as loss of dexterity, falls or trips. Speech or swallowing problems, or tongue fasciculations (this is known as bulbar presentation). Muscle problems, such as weakness, wasting, twitching, cramps and stiffness. Breathing problems, such as shortness of breath on exertion or respiratory symptoms that are hard to explain. Effects of reduced respiratory function, such as excessive daytime sleepiness, fatigue, early morning headache or shortness of breath when lying down. MND may first present with cognitive features, which may include: Behavioural changes. Emotional lability (not related to dementia). Frontotemporal dementia. Lower motor neurone dysfunction in the limbs manifests as weakness, atrophy, fasciculations and hyporeflexia. The thighs are often a site of marked fasciculation. Fasciculation can be difficult to distinguish from arterial pulsation, so consider if there is an underlying arterial course before defining twitching movements as fasciculation. Upper motor neurone dysfunction manifests as weakness predominating in the arm extensors and leg flexors with evidence of hypertonia, hyperreflexia and upgoing plantar responses; the bulbar muscles may also show spasticity with an exaggerated jaw jerk. Ocular, sensory or autonomic dysregulation signs are usually late features of the disease. Riluzole (a neuroprotective glutamate-release inhibitor) is the only drug of proven disease-modifying efficac