Pathology 1 Flashcards
Multiple Myeloma /plasmacytoma (singular)
Multiple myeloma is a rare cancer characterized by excessive proliferation and improper function of plasma cells found in the bone marrow.
The most common symptom is bone pain, usually of the lower back or ribs. Susceptible to fracture, collapse and potentially cord compression. That would give pain/weakness/numbness in limbs. May also have hypercalcaemia. Weak immune system, kidney dysfunction, anaemia and bleeding.
Investigations include serum protein electrophoresis for Igm. Bone marrow aspiration and biopsy gives prognosis. Whole body MRI first line imaging.
First line treatment is Bortezomib and Thalidomide with consideration for stem cell transplant. If acute renal disease with it try bortezomib with dexamethasone. For bone health, provide zoledronic acid to reduce fractures.
Polyarteritis Nodosa
Blood vessel disease with inflammation of small and medium-sized arteries (vasculitis), preventing them from bringing oxygen and food to organs for those middle-aged. PAN most commonly affects vessels related to the skin, joints, peripheral nerves, gastrointestinal tract, heart, eyes, and kidneys.
Main symptoms include kidney issues, arthralgia, elevated CRP, fever and myalgia, rashes, numbness, abdominal pain. Idiopathic or Hep B/C.
Diagnose with artery biopsy.
Treatment recommendations are prednisone PO but if multiple mononeuritis give methyl prednisolone. Along with this give cyclophosphamide.
If Hep, try to give just antiviral. If HTN give ACE as well.
Cushing’s Syndrome/Disease
Hypercortisolemia in Cushing’s syndrome is usually due to a corticotropin (ACTH)-producing pituitary tumor (Cushing’s disease), ectopic ACTH secretion by a nonpituitary tumor, or cortisol secretion by an adrenal adenoma or carcinoma. Can be syndrome from the overuse of corticosteroids.
Symptoms include a “buffalo hump”, a red puffy rounded face, large purple stretch marks
weakness in your upper arms and thighs,
a reduced sex drive (low libido) and fertility problems
depression and mood swings.
Urinalysis, FBC, saliva tests.
Treatment before surgery/radiotherapy is Metyrapone (alone if CS or DM) and Ketoconazole (for CD) as adrenal enzyme inhibitors. For a pituitary tumour use cabergoline.
Polycythemia Vera
Cancer of red blood cells in the bone marrow. Key symptoms are headaches, blurred vision, red face and distal limbs, HTN, fatigue, confusion, excessive bleeding, gout and itchy skin particularly after water contact.
Risk of PE, DVT and other clots. Test by FBC. Treated by venesection + hydroxycarbamide or interferon to reduce rbc production.
Gestational HTN-HELLP Syndrome
When Gestational HTN 140-160 becomes preeclampsia with protein urea and/or liver/kidney impairment (with seizures becomes eclampsia) that progresses to Haemolysis, elevated Liver enzymes and Low Platelets/HELLP syndrome.
For HTN- initially give labetalol or nifedipine. If progresses to preeclampsia give magnesium sulphate to avoid seizure. Preeclampsia also give methyldopa and labetalol. If progresses to HELLP, don’t give other corticosteroid than Dexamethasone. Delivery is also a cure for all if safe.
Immune Thrombocytopenic Purpura
Immune thrombocytopenic purpura (ITP) is a blood disorder characterized by a decrease in the number of platelets in the blood. Can cause easy bruising, bleeding gums, and internal bleeding.
Acute thrombocytopenic purpura. This usually affects young children, ages 2 to 6 years old. The symptoms may follow a viral illness, such as chickenpox. Acute ITP usually starts suddenly and the symptoms usually disappear in less than 6 months, often within a few weeks. Treatment is often not needed.
Chronic thrombocytopenic purpura. The onset of the disorder can happen at any age, and the symptoms can last a minimum of 6 months for teenagers and adults. Females have it more often than males.
Symptoms include red/purple petechial rash, nosebleeds, fatigue, heavy menstruation, blood in vomit, urine or stool.
Treatment for chronic is prednisolone and maybe IV antiD. If persists, give Caplacizumab.
Aspergillosis
Aspergillosis is a condition caused by breathing in aspergillus mould. Can come from soil, plants, damp buildings and aircon systems. Usually only at risk if have immunosuppression or asthma/CF/COPD.
Symptoms are short of breath, cough over 3 weeks with or without blood/mucous, fever, weight loss, aspergilloma kidney bean shape on CXR.
Treatment is Itraconazole or if invasive, amphotericin B.
Karposi’s Sarcoma
Angioproliferative cancer that requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated herpes virus (KSHV), for its development . KS is classified into four types: classic/Mediterranean (typically presents in middle or old age), endemic/Sub-Saharan, iatrogenic (a type associated with immunosuppressive drug therapy, typically seen in renal allograft recipients), and AIDS associated (epidemic KS).
Characterised in Mediterranean or sub-Saharan Africa. Has lesions that are purple red or dark brown macules, plaques and nodules on the skin that ulcerate and bleed. Give a biopsy. Other symptoms include lymphoedema, chest pain, coughing blood, nausea and GI upset.
Treatment can involve scraping, radiotherapy and Paclitaxel. If HIV associated, give the drug Daunorubicin.
Lymphoma (NHL)
Non-Hodgkin lymphoma, which is the most common type, typically develops from B and T lymphocytes (cells) in the lymph nodes. Some people will not experience any symptoms. Nodes may swell, weight loss, fever, itching, fatigue, painful nodes after alcohol. Mostly people 60 yrs +. (Hodgkin’s is for 20-30 years old. and DBV makes more likely. The symptoms are similar)
Treatment is Rituximab. For Hodgkin’s it is ABVD (Doxorubicin, Bleomycin, Vinblastine, Dacarbazine).
Granulomatosis with Polyangiitis (Wegener’s) or ANCA associated vasculitis.
Granulomatosis with polyangiitis is an uncommon disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Risk of necrosis can be fatal.
Symptoms include fatigue, fever, weakness, weight loss, nosebleeds or blocked nose, earache, cough, shortness of breath, wheezing, glomerulonephritis, rashes and dots, double vision, GI upset.
Investigations include ANCA blood test, CT or MRI, Biopsy.
Treatment of choice is Cyclophosphamide or Rituximab until stable, then maybe a long term immunosuppressant like Methotrexate or Azathioprine,.
Sarcoiodosis
Sarcoidosis is a rare condition that causes small patches of red and swollen tissue, called granulomas, to develop in the organs of the body. It usually affects the lungs and skin.
The symptoms of sarcoidosis depend on which organs are affected, but typically include:
tender, red bumps on the skin, particularly shins
shortness of breath
a persistent dry cough
For some people, it returns and becomes chronic. Xray or biopsy diagnoses.
Treatment is prednisolone and infliximab if needed.
Wilson’s Disease
Wilson disease is a rare genetic disorder characterized by excess copper stored in various body tissues, particularly the liver, brain, and corneas of the eyes. The disease is progressive and, if left untreated, it may cause liver (hepatic) disease, central nervous system dysfunction, and death.
Presents with jaundice and symptoms of liver disease,. Younger patients may have rusty brown Kayser-Fleischer rings in the corneas. No menses for females.
Treatment is a combo of penicillamine for copper chelation( or trientine dihydrochloride), zinc salts to stop absorbsion and tetrathiomolybdate to bind in blood to reduce toxicity
Hereditary Haemochromatosis
Inherited iron overload for those of white background most commonly. Usually between ages 30-60.
Symptoms include fatigue, weight loss, weakness, joint pain, erectile dysfunction, no menses, jaundice, abdo/thorax pain, swelling of hands and feet, testicles shrinking. Diagnosed via blood test.
Treatment with repeated venesection. Where not good, desferrioxamine mesilate IV is given sometimes with ascorbic acid/vitC.
Sinusitis
Sinusitis is defined as symptomatic inflammation of the paranasal sinuses.
Acute sinusitis is usually triggered by a viral upper respiratory tract infection and is defined by symptoms that last for less than 12 weeks.
Chronic sinusitis is symptoms that last over 12 weeks with sinonasal inflammation.
In adults, it is diagnosed by the presence of nasal blockage or nasal discharge with facial pain/pressure (or headache) and/or reduction of the sense of smell.
In children, it is diagnosed by the presence of nasal blockage or discoloured nasal discharge with facial pain/pressure and/or cough.
Examination may reveal the presence of purulent discharge, mucosal oedema, tenderness over the sinuses, and fever.
If acute and has over 10 days wi/o improvement give antibiotics like phenoxymethyl penicillin or doxycycline and intranasal corticosteroids like mometasone.
If chronic give saline irrigation and intranasal corticosteroids like mometasone or fluticasone.
Addison’s Disease/hypoadrenalism/primary adrenal insufficiency
Addison’s disease (primary adrenal insufficiency) is caused by destruction of the adrenal cortex. This results in reduced production of glucocorticoids (such as cortisol), mineralocorticoids (such as aldosterone), and adrenal androgens (such as dehydroepiandrosterone).
Adrenal insufficiency may be caused by long-term administration of corticosteroids or disorders of the hypothalamus or pituitary gland, but this is not Addison’s disease. Mostly autoimmune, but can be CAH or TB.
Symptoms include fatigue, loss of appetite, hyperpigmentation, salt cravings, nausea, abdominal pain, polydipsia, puberty delay, irregular periods, loss of pubic hair, hypoglycaemia.
A person with Addison’s disease may present with a sudden crisis precipitated by intercurrent illness or other stress. Features include hypotension, hypovolaemic shock, acute abdominal pain, vomiting, and reduced level of consciousness. Give immediate hydrocortisone.
If low in cortisol, treated by glucocorticoid replacement with hydrocortisone three times a day.
If low in aldosterone, give mineralocorticoid replacement by fludrocortisone.
If androgens, give DHEA replacement.
