Pathology 4

Question 1

Leukaemia: common features

Answer 1

Common features include diffuse replacement of normal bone marrow by leukaemic cells with variable accumulation of abnormal cells in the peripheral blood.

Infiltration of liver, spleen, lymph nodes, meninges and gonads.

Bone marrow failure with anaemia, neutropenia and thrombocytopenia.

Leukaemia can be stimulated by ankylosing spondylitis, alkylating agents for lymphomas, benzene exposure, Down syndrome, viruses.

Question 2

Acute lymphoblastic leukaemia

Answer 2

Most common between 2-4 years. For some the mutations occur in uterus with further mutations following birth. Majority of them derive from B cell precursors. Antibodies give indication of their maturity. Includes Philadelphia chromosome. Med neg means less likely to relapse.

Question 3

Acute lymphocytic leukaemia

Answer 3

Mostly B lymphocytes. Is 80% of leukaemias in children. A small number of over 50s. Shows rapid deterioration, fatigue, dizziness, palpitations, severe bone and joint pain, recurrent and severe infections, fevers, splenomegaly, dyspnoea, abdominal mass, testicular enlargement, gum hypertrophy, cranial nerve palsy 3,6 and 8 if disease is mature as Burkitts leukaemia.

Regime vincristine, methotrexate and athracycline, maybe cyclophosphamide

Question 4

Acute myeloblastic leukaemia

Answer 4

Often accumulates in liver and spleen from failed apoptosis. Raised WBC’s, neutropenia, fever, bleeding or dic from thrombocytopenia. If WBC’s too low can get respiratory distress.

Includes bone pain, liver, spleen, gingivitis. Treatment includes cytarabine and daunorubicin. Post remission give daunorubicin, mitoxantrane or arabinosylcytosine. Sometimes use ATRA with chemo if APC AML.

Children are more likely to achieve remission than adults.

Question 5

Chronic myeloid leukaemia

Answer 5

Median survival 5 yrs without stem cell transplant. Occurs in all age groups. Healthy marrow is replaced by stem cells of erythoid, megakaryotic, granulocyte and Bcell lineage. Can go into an aggressive phase called blast crisis.

Fatigue, night sweats, weight loss, luq pain from splenic infarction, splenomegaly, hepatomegaly, anaemia, easy bruising, gout, fever, hyper viscosity syndrome. Treat with imatinib and stem cell transplant.

Scts can risk graft vs host disease, venoocclusion, infection, secondary malignancies.

Question 6

Chronic lymphocytic leukaemia

Answer 6

Accumulation of Bcells. Usually less aggressive and affects the elderly. Median survival is 25 yrs and often doesn’t require treatment. Can get an aggressive form shortening it to 8/9. Anemia and thrombocytopenia are late developments. Advanced NHL can look like CLL. Nodes, liver and spleen can be affected = small cell lymphocytic lymphoma.

1/4 of all leukaemia, symmetrical lymphadenopathy, pneumonia and herpes common. Spleno/hepatomegaly and abdominal pain, pallor and tonsillar enlargement.

Chemo is cyclophosphamide or chlorambucil. Could pair cyclophosphamide with fludarabine and add rituximab sometimes. Stage A survival 10 yrs, stage C is 18 months. If have p53 deletion, prognosis is worse.

Question 7

Chlamydia Trachomatis

Answer 7

Infects urethra, endocervix, rectum, pharynx, conjunctiva. Contact/fluid transmission. Up to 80% women and 50% men can be asymptomatic. Can complicate to pelvic inflammatory disease that can give tubal infertility, ectopic pregnancy and chronic pelvic pain.

Incubation period 7-21 days. Men = anterior urethritis with mucal discharge worse on waking with crusting at meatus and dysuria. If goes along vas deferens can lead to epididymorchitis.

Women show endocervical symptoms, vaginal discharge, dysuria, postcoital bleeding and lower abdominal pain. Examination of the cervix may reveal mucopurulent cevicitis and/or contact bleeding. Ascending infection into uterus and Fallopian tubes leading to endometriosis and acute salpingitis (PID).

In pregnancy, associated with preterm birth, postpartum infection, neonatal mucopurulent conjunctivitis and pneumonia via vaginal delivery. Vertical transmission.

Rectal infection via anal sex and be asymptomatic or proctitis. Reactive arthritis if hla b27 positive.

One ceftriaxone 500mg IM with doxycycline twice daily and metronidazole twice daily for two weeks. No sex.

Question 8

Epididymo-orchitis

Answer 8

Pain and swelling of epididymis via extension of urethral or bladder infection. Can be c trachomatis, n gonorrhoeae or a UTI. Most common differential is spermatic cord torsion.

Subacute onset of unilateral scrotal pain and swelling. May be urethral discharge or dysuria. Tender and palpable swelling of epididymis and maybe testicle and erythema of scrotum.

If STI give eg ceftriaxone and doxycycline. UTI ofloxacin x2 daily for 2 weeks.

Question 9

Bacterial vaginosis

Answer 9

Lactobacillus dominant flora replaced by other bacteria like gardnerella vaginalis, anaerobes, mycoplasmas and mobilunous. Not an std.

In pregnancy associated with risk of miscarriage and preterm birth. Increases risk of getting and spreading HIV.

Fishy odour and increased discharge. Creamy white with froth, can adhere to vaginal walls. No vaginal inflammation.

Diagnose with microscopy of gram stained discharge must have ph > 4.5. Treat with metronidazole x2 daily for a week. Cream for five nights if pregnant.

Question 10

Candidiasis

Answer 10

Candida albicans. Not an STI. Higher risk of infection if pregnant or diabetic. 75% women have it at least once. Can increase risk with broad spectrum antibiotics and corticosteroids. Men can contract via sex and have a rash.

Vulval itching/burning, thick white discharge, external dysuria and superficial dyspareunia. May see vulval erythema, fissuring and oedema.

Itraconazole twice in a day, or one dose fluconazole with clotrimazole cream.

Question 11

Trachomoniasis vaginalis

Answer 11

Most common global STI. Flagellates protozoan. In women it infects vagina and urethra. Men urethra and supraprepubital sac. Men are often asymptomatic. In pregnancy there’s risk of preterm birth and low birth weight. Increased risk of HIV acquisition.

Vaginal purilent smelling discharge with possible vulval pruritus/dysuria and dyspareunia. May be erythema, discharge is yellow or grey, frothy and profuse. Cervix might have small haemorrhages - strawberry cervix.

Treat with metronidazole twice a day for a week.

Question 12

Gonorrhoea

Answer 12

STI of neisseria gonorrhoeae. Spread via discharge with sex etc. It can infect the cervix, urethra, rectum and facial orifices. Can be transferred to a baby in utero and cause permanent blindness.

Thick yellow or green discharge, painful urination and intermenstrual bleeding.

Treat with one time ceftriaxone and azithromycin.

Question 13

Human papilloma virus/ anogenital warts.

Answer 13

HPV 6 and 11. Painless benign epithelial tumours. Skin contact transmission. Neonates may get HPV from infected birth canal = anogenital warts or laryngeal papillomatosis.

Incubation period 3 months and appears at site of sexual contact first. Prepuce, glans then to urethra, penile shaft. forchette to vulva and perineum.

Warts on mucous membranes tend to be soft and non keratinised. On hair bearing skin likely to be firm and keratinised.

Treating non keratinised = topical podophyllotoxin. Keratinised gets ablation via cryotherapy or electrocautery. Imiquimod helps for both types. If pregnant, hiv or immunosuppressive just ablaze.

Vaccinations at 12= 1 dose for 16 and 18 then another for 6, 11, 16 and 18.

Question 14

Molluscum contagiosum

Answer 14

Large DNA virus leading to 2-5mm diameter benign smooth papules with central umbilication. Spread via skin contact. When sexual, lesions are multiple, on labia majora, penile shaft, pubic region, lower abdomen and upper inner thighs.

Often self limiting and resolves naturally, can treat with same as HPV anogenital warts.

Question 15

Herpes Simplex/Genital herpes

Answer 15

Most universal cause of genital ulcers. Transmission is via mucous membrane of a person who is shedding the virus and can be asymptomatic. Genital herpes is usually due to HSV1 or 2. Can be both.

During primary infection the virus ascends peripheral sensory nerves and establishes latency in the dorsal root ganglia.

Multiple sore shallow ulcers, inguinal lymphadenopathy and systemic symptoms of viraemia, eg fever, myalgia and headache. Women can have dysuria and vulval pain. Ulcers on cervix can look like malignancy. Rectal ulcers can lead to proctitis, pain and bleeding. Can complicate to aseptic meningitis.

Reactivation makes tingling, itchy or pain in area. Treat with salt water bathing and aciclovir.

Question 16

Syphilis

Answer 16

Via motile spirochaete treponema palidium. Spread via direct contact with lesion and enters new host via breaches in skin or mucosa, usually during sex. Can be maternally transmitted in utero so can be congenital or acquired. Latter is divided into primary and secondary.

Primary= ~21 days past exposure a papule develops at site and ulcerated to a firm chancre with painless regional lymphadenopathy that heals within 2-6 weeks.

Secondary= 6-10 weeks after primary lesion. Fever, sore throat, malaise and arthralgia. Widespread rash- coppery and maculopapular on palms and soles too. Condylomata lata = wart like plaques in the perianal area and moist body sites. Can become latent. If untreated can come back granulomatous and is destructive to skin and bones, heart, cns.

Treat with benzathine penicillin G or doxycycline. Often experience reactive flu symptoms = jarisch herxheimer reaction.

Question 17

Perthe’s Disease

Answer 17

Rare childhood condition affecting the hip. Occurs when the blood supply to the head of femur is temporarily disrupted leading to some avascular necrosis, weakening the bone over years until it collapses. Over time, the blood supply returns and the bone begins to grow back.

Your child may limp, have limited motion, or develop a peculiar running style, all due to irritability within the hip joint. Other common symptoms include:

Pain in the hip or groin, or in other parts of the leg, such as the thigh or knee (referred pain).
Pain that worsens with activity and is relieved with rest.
Painful muscle spasms that may be caused by irritation around the hip.

Question 18

Erythema infectosum/slapped cheek syndrome/fifth disease

Answer 18

Erythema infectosum is caused by an erythrovirus, EVB19 or Parvovirus B19. It is a single-stranded DNA virus that targets red cells in the bone marrow. It spreads via respiratory droplets, and has an incubation period of 7–10 days.

Parvovirus B19 infection causes nonspecific viral symptoms such as mild fever and headache at first. The rash, erythema infectosum, appears a few days later with firm red cheeks, which feel burning hot. This lasts 2 to 4 days, and is followed by a pink rash on the limbs and sometimes the trunk. This develops a lace-like or network pattern.

Although most prominent in the first few days, the rash can persist for up to six weeks at least intermittently, and is most obvious when warm.

Question 19

VDRL and FTA (and FTA ABS) tests

Answer 19

Used to detect antibodies to the bacteria Treponema pallidum, which causes syphilis

Question 20

What is Bence Jones protein?

Answer 20

A Bence Jones protein is a monoclonal globulin protein or immunoglobulin light chain found in the urine, with a molecular weight of 22–24 kDa. Detection of Bence Jones protein may be suggestive of multiple myeloma or Waldenström’s macroglobulinemia.

Question 21

Hirschsprung disease/congenital megacolon

Answer 21

Hirschsprung disease (HSCR) is a birth defect. This disorder is characterized by the absence of particular nerve ganglions in a segment of the bowel in an infant. The absence of ganglion cells causes the muscles in the bowels to lose peristalsis. Affected individuals can develop constipation and partial or total obstruction of the bowels. Pain and discomfort can result. If not treated, a potentially serious bacterial infection may develop. Requires bowel resection.

Symptoms in the newborn period include failure to pass the meconium within a short time after birth. Failure to pass a first stool for 24-48 hours is suggestive of HSCR.

Infants with HSCR will very often have abdominal swelling (distention), abdominal pain, and vomiting. Affected infants have constipation and often exhibit poor weight gain, and slow growth.

HSCR can sometimes lead to a condition called enterocolitis, which is inflammation of the small intestines and colon. This is often referred to as Hirschsprung-associated enterocolitis.

Hirschsprung-associated enterocolitis often presents with fever, explosive diarrhoea, abdominal swelling, lethargy, and vomiting. Some individuals with either severe or untreated Hirschsprung-associated enterocolitis may develop sepsis.

Question 22

Superior sagittal sinus thrombosis

Answer 22

Superior sagittal sinus thrombosis (SSST) is the most common type of dural venous sinus thrombosis and is potentially devastating.

Risk factors include pregnancy, dehydration, hypercoagulable states, pancreatitis.

Clinical presentation
As with all cerebral venous thrombosis, the presentation is highly variable, ranging from completely asymptomatic to a rapid fulminant course with cerebral haemorrhage and death.

Presentation includes:
headache: 53%
seizures: 48%
hemi-, quadri-, or paraplegia: 48%
visual disturbances: 25%
nuchal rigidity: 18%

Might see a cord sign on CT, haemorrhage, cerebral oedema.

Question 23

Antibiotics for Bites

Answer 23

Dog = amoxicillin-clavulanate (or clindamycin and fluoroquinolone/trimethoprim-sulfamethoxazole)

Cat = amoxicillin-clavulanate (or cefuroxime/doxycycline)

Human = amoxicillin-clavulanate (or clindamycin and ciprofloxacin/ trimethoprim-sulfamethoxazole)

Question 24

Haloperidol

Answer 24

Haloperidol is an antipsychotic medicine that is used to treat schizophrenia. Haloperidol is also used to control motor and speech tics in people with Tourette’s syndrome.

Side effects include dry mouth.
increased saliva.
blurred vision.
loss of appetite.
constipation.
diarrhoea.
heartburn.
nausea.

Question 25

Cefotaxime for children

Answer 25

Third generation cephalosporin antimicrobial agent which has received wide acceptance as a first-line antibiotic treatment of various serious bacterial infections in paediatric patients, primarily meningitis and sepsis.

Question 26

Tobramycin

Answer 26

Mostly topical for eye infections.

Other uses: Septicaemia,
Meningitis and other CNS infections,
Biliary-tract infection,
Acute pyelonephritis or prostatitis,
Pneumonia in hospital patients and UTIs.

Question 27

Ceftazidime

Answer 27

A cephalosporin used for prostatic resection prophylaxis, pseudomonas lung infections in CF, complicated UTIs, septicaemia/HAP, febrile neutropenia, meningitis.

May cause abdominal pain or diarrhoea.

Question 28

Infliximab

Answer 28

Treatment for crohns disease, ulcerative colitis, rheumatoid arthritis, sometimes for ankylosing spondylitis, psoriatic or plaque arthritis.

Contraindicated with heart failure, severe infections. Side effects can be anything.

Question 29

Ceftriaxone for paediatrics

Answer 29

Ceftriaxone has a broad spectrum of in vitro activity and is used in neonates for treating gonococcal ophthalmia and as a second line drug for sepsis and meningitis. It can precipitate if used together with calcium leading to severe reactions including neonatal death.

Question 30

Currarino triad

Answer 30

Currarino triad or syndrome is an autosomal dominant hereditary condition which is characterized by the triad of sacral agenesis abnormalities (abnormally developed lower spine), anorectal malformation (most commonly in the form of anorectal stenosis) and presacral mass consisting of a teratoma, anterior sacral meningocele or both.

Commonly an autosomal dominant mutated MNX1 gene. Might have an undersized sacrum, scrotal cleft, teratoma, arteriovenous malformation or intestinal abnormalities.

Question 31

Rhinovirus

Answer 31

Rhinovirus is the main culprit behind the common cold. Illness usually starts with a runny nose, sore throat, and sneezing and may go on to include headache, cough, and muscle aches. Most rhinovirus infections are mild, but they can sometimes lead to bronchiolitis and pneumonia – especially in babies.

Question 32

Enterovirus

Answer 32

Group of single-stranded sense RNA viruses that commonly cause infections, especially in infants and children. They are responsible for a myriad of clinical syndromes including hand-foot-and-mouth (HFM) disease, herpangina, myocarditis, aseptic meningitis, and viral chest pain.

Mild symptoms of enterovirus infection may include fever, runny nose, sneezing, cough, skin rash, mouth blisters, and body and muscle aches. Children with asthma are particularly at risk for severe symptoms from enterovirus infection.

Question 33

Adenovirus

Answer 33

Adenoviruses cause acute respiratory disease (usually), pneumonia (occasionally), acute follicular conjunctivitis, epidemic keratoconjunctivitis, cystitis, and gastroenteritis (occasionally). In infants, pharyngitis and pharyngeal-conjunctival fever are common.

Symptoms:
common cold or flu-like symptoms.
fever.
sore throat.
acute bronchitis 
pneumonia 
pink eye

Question 34

Capillary hemangioma

Answer 34

A capillary hemangioma (“strawberry” birthmark) is a benign tumour consisting of an abnormal overgrowth of tiny blood vessels. Capillary hemangiomas may not be present at birth, but appear within the first 6 months of life. Can be troublesome if on the eyelid or around areas of groin friction.

Question 35

Haemolytic uremic syndrome (HUS)

Answer 35

The combination of:
Microangiopathic haemolytic anaemia (Coombs’ test negative).
Thrombocytopenia.
Acute kidney injury (acute renal failure)

It’s the most common cause of acute kidney injury in children. It’s commonly associated with Escherichia coli that has a shiga toxin causing damage to renal, GI and CNS .

The classical presenting feature is profuse diarrhoea that turns bloody 1 to 3 days later. Half have fever before admission and painful abdomen/vomiting. Pain on bowel movement.

High lactase dehydrogenase is an early sign.

Question 36

Palivizumab

Answer 36

Is used to prevent serious LRTI disease from RSV and/or in the circumstance of a vulnerable child to LRTI in cardiac bypass surgery.

Can cause apnoea.