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Nigerian National Diagnostic Histopathology EQA circulation 007 (For the Nov 2014 TSL workshops). Clinical information Please submit your answers online via the EQA page of www.tslworkshopsng.com. The submission of responses is crucial to the overall quality of the program Part 1: Autopsy Pathology There are 3 cases in this section (PM1, PM2 and PM3). Key slides have been provided for each case and there is a summary of the PM report for each case (attached as an appendix) at the end of this document. For each case A) identify the organ on the slides and state the histologic abnormalities B) Putting the findings in A together with the information in the PM report, propose a primary disease and cause of death. Part 2: Lymph node/lymphoma pathology (emphasis on the use of immunohistochemistry) 1) 3 year old boy with bilateral neck swelling and recurrent cough x 8 months. There was also night sweats and progressive weight loss. Neck lymph node excision biopsy IMMUNOHISTOCHEMISTRY: The lesional cells express S100 but are negative with CD1a, melan A and broad-spectrum cytokeratins (AE1/3). 2) 67 year old man with generalised lymphadenopathy. IMMUNOHISTOCHEMISTRY: The lesional cells express CD20, CD5 and CD23 with a Ki-67 index that ranges from 5% to 30% in different areas 3) 27 year old female with generalised lymphadenopathy ? cause. Excision biopsy of neck lymphnode IMMUNOHISTOCHEMISTRY: Lesional cells negative with CD45 and EBVLMP1, positive with CD15 and CD30. 4) 87 year old male with left haemorrhagic pleural excision and bilateral axillary lymph nodes. Lymph node biopsy IMMUNOHISTOCHEMISTRY: Lesional cells strongly express CD20 and CD23 but are negative with CD5. Ki-67 index is focally up to 10% in areas. 5) Supraclavicular lymph node 28 yr old female who is very ill with multiple enlarged lymph nodes, distended neck veins and collapsed/consolidated right lung. IMMUNOHISTOCHEMISTRY: Lesional cells express CD30 but not CD15. They are also negative for CD45, EBV-LMP1, CD3, ALK-1, CD20 and EMA. 6) Male 13 years, biopsy site not stated. Clinically ? Rhabdomyosarcoma IMMUNOHISTOCHEMISTRY: lesional cells strongly and uniformly express CD30, CD45, CD4 and EMA with weak focal expression of CD8. They are negative with CD15, AE1/3, CD3, CD5, PAX5, CD20, EBV-LMP1, S100, Desmin and Sarcomeric actin. 7) Male 21 yrs.Multiple generalised masses (lymphadenopathy) - cervical, axillary, preauricular, occipital, inguinal and chest wall for 3 months. Hilar lymphoma reported on chest x-ray. Patient says masses suddenly began to shrink after growing to a large size. Diagnosis - non-Hodgkin's lymphoma probably lymphoblastic lymphoma IMMUNOHISTOCHEMISTRY: There is some difficulty with immunohistochemistry due to poor original preservation, but there is definite strong positive staining with TdT and CD10. Bcl-2 also appears positive as does CD5. CD20 marks a small apparently residual population of nodal B lymphocytes. There is no expression of cyclin D1 or CD23. The Ki-67 index of the tumour cells is up to 40%. 8) 44 yr old female with axillary lumps. IMMUNOHISTOCHEMISTRY: Lesional cells express CD20, CD10 and Bcl-2 but not CD5 9) Left neck lymph node enlargment of 3 months duration in a 30 yr old female IMMUNOHISTOCHEMISTRY: No fungal or mycobacterial organism identified with special stains and EBV-LMP1 negative. 10) 52 year old woman with swelling on the forehead IMMUNOHISTOCHEMISTRY: Not yet done 11) 74 year old man with long history of chronic granulomatous dermatitis. Enlarged left supratrochlear lymph node biopsied IMMUNOHISTOCHEMISTRY: Not yet done SEE APPENDIX FROM NEXT PAGE EXTRACTS FROM POST MORTEM REPORTS PM1 CLINICAL HISTORY The index illness started with vomiting after during a meal with the husband. The vomiting continued intermittently and was later compounded by breathlessness. There was no diarrhoea. She was given IV infusions and some drugs including hydralazine and aminophylline during an admission in hospital on the same day. The systolic blood pressure was initially 200mmHg but later dropped to 120mmHg after medications. The patient’s condition worsened and she was transferred to another hospital a few days later where she was diagnosed with shock. She died within 24 hours of admission in the second hospital. EXTERNAL EXAMINATION Middle aged female with dark maculo-papular skin rashes on the entire body. The body was moderately dehydrated. There was neither palor nor jaundice. There was also no sign of trauma on the body. There were sutured incisions above the medial area of both ankles (which were said to be areas of venous access [cut-down]). INTERNAL EXAMINATION The visceral organs were in their normal anatomical positions. There was no excess fluid or blood in the cavities. There was no sign of trauma. Brain: 1230g. NAD Trachea and Bronchial Tree: NAD Right Lung: (640g) The right lung was heavy and sub-crepitant with patchy variable sized haemorrhagic sub-pleural bullae. No sign of aspiration was seen. Cut sections revealed very congested and oedematous surfaces, oozing blood and frothy fluid. Cut pieces from all lung lobes floated in water. Left Lung: (604g). The left lung was heavy and sub-crepitant with patchy variable sized haemorrhagic sub-pleural bullae. No sign of aspiration was seen. Cut sections revealed very congested and oedematous surfaces, oozing blood and frothy fluid. Cut pieces from all lung lobes floated in water. Heart and Great Vessels: NAD. Heart 250g Tricuspid valve circumference 13.0cm, Pulmonary valve circumference 7.4cm Mitral valve circumference 9.5cm Aortic valve circumference 7.0cm Oesophagus: NAD Stomach: There was obvious flattening of the rugal folds and also patchy haemorrhagic lesions on the mucosal surface. Small Intestine: NAD Large Intestine: The large bowel contained some dark fecal matter. Liver and Gall Bladder: The liver (1340g) capsule was normal. However, the sub-capsular surfaces show patchy light yellowish discoloration. Cut sections show moderate fatty changes and congestion. The gall bladder contained bile and the duct was patent. Spleen: The splenic capsule was wrinkled. Cut section of the spleen was fairly normal. Pancreas: NAD Right Kidney and Adrenal: The right kidney weighed 100g. The capsule stripped with ease. Cut surface showed global palor with hyperamia in the cortico-medullary border. The right adrenal was anatomically normal. Left Kidney and Adrenal: The left kidney weighed 100g. The capsule stripped with ease. Cut surface show global palor with hyperamia in the cortico-medullary border. The left adrenal was anatomically normal. Urinary Bladder and Ureters: NAD Uterus and adnexia: NAD PM 2 DOD- 19/2/14 DOP- 20/2/14 O.F., 57 year old Brine discharge operator, known RVD patient (on ?HAART) referred from the Navy Reference Hospital on account of fever of 2 days’ duration, and vomiting of blood and loss of consciousness of 5 hours’ duration. Fever has been occurring on and off for some time (the exact duration was not stated) and he has been receiving treatment at the Navy Reference Hospital. Fever was said to be high grade and associated with chills. The pattern of the fever is not documented. Five hours prior to presentation, the patient started vomiting blood. After 2 episodes of vomiting frank blood, he lost consciousness. He has a positive history of associated reversal of sleep pattern. No history of patient being irritable or acting aggressive. He had hepatitis 7 years ago. There is positive history of significant alcohol ingestion. There is positive history of progressive abdominal and leg swelling. There is no orthopnea, paroxysmal nocturnal dyspnoea. He was not a known hypertensive or diabetic. On clinical examination, he was found to be a middle aged man, unconscious (GCS- 3/15), afebrile (T-34.82oC), severely pale, severely icteric, and bilateral pitting pedal oedema up to the distal third of the leg. He had no pubic or axillary hair. The abdomen was uniformly distendedby fluid. Ascites was demonstrable by fluid thrill. The liver was shrunken (Liver span- ?5cm), the spleen and the kidneys were not palpably enlarged. Digital rectal examination showed anal tags on the verge, and gloved finger was stained with dark stools The pulse rate-105beats/ min, BP- 83/40mmHg, heart sounds 1 and 2 only, RR- 30 cycles/ min, vesicular breath sounds. Clinical diagnosis: 1. Coma secondary to decompensated chronic liver disease precipitated by sepsis 2. Upper GI bleeding possibly due to ruptured oesophageal varices His condition continued to deteriorate and he died about 2 hours after he was admitted. POSTMORTEM FINDINGS We received the body of a cachectic middle-aged man who is severely pale, deeply icteric with bilateral pitting pedal oedema. Serous cavities The peritoneal cavity contains 1.5L of serous fluid, the pleural cavity about 200mls of serous fluid. The pericardial cavity is free of excess fluid. Trachea, bronchi, lungs and pleura Both lungs are heavy, the right and the left lungs weigh 600 grams and 500 grams respectively. All lung lobes feel subcrepitant to touch. Cut sections through all lobes show free flowing frothy fluid. Floatation test is negative. The pulmonary vasculature is free of thrombi. Heart, pericardium and great vessels The heart is not enlarged, it weighs 350 grams. The heart measurements are as follows: All the valvular circumferences are within normal limits. The left ventricular free wall measures 1.6cm in thickness while the right ventricular free wall measures 0.5cm in thickness. The papillary muscles appear bulbous. The coronary vessels are patent. No thromboemboli in the pulmonary trunk. Oesophagus, stomach and intestines An ulcer is seen on the mucosa of the distal oesophagus. 200mls of fresh blood is found in the stomach. Liver and Gallbladder The liver is not enlarged, it weighs 1250 grams. Cut sections show multiple macro and micronodules diffusely distributed throughout the parenchyma. The spleen is enlarged and weighs 500 grams. Cut sections show prominent fibrous trabeculae. Kidneys Both weigh 100grams. Cut sections show increased corticomedullary differentiation. Other organs appear unremarkable. PROVISIONAL ANATOMIC SUMMARY Retroviral disease (status clinical) with Severe anaemia and shocked kidneys Upper Gastrointestinal bleeding Ruptured oesophageal varices Dilated oesophageal veins due to Portal hypertension Liver cirrhosis Chronic liver disease with severe jaundice PM 3 B. A., 42 year old female, known patient with depression who is being managed at the Neuropsychiatric Hospital, and who was referred on account of abdominal pain and vomiting of 4 days, and constipation of 2 days’ duration. The abdominal pain was of acute onset, colicky, and associated with abdominal cramps. The pain progressively became generalized. Vomiting was non-projectile, she had up to 10 episodes/ day and vomitus consisted of recently ingested foods. She developed absolute constipation 2 days later. She had Caesarean section 5 years before the present illness. No chronic illnesses or other significant information in her past medical history. On examination, she was found to be drowsy (GCS-not stated), febrile (Temperature-not stated), moderate pallor, no jaundice, no cyanosis, no peripheral lymphadenopathy, no pedal oedema. Pulse rate, blood pressure and RR are normal. Examination of the abdomen revealed a distended abdomen (Abdominal girth-not stated), with multiple scarification marks, healed Pfannenstiel scar, moderate tenderness, nil rebound tenderness, nil rigidity, reduced bowel sounds. Digital rectal examination showed an anal tag at the 11 o’clock position, normal sphincteric tone, rectum filled with loose faeces. Clinical diagnosis: Small intestinal obstruction, likely secondary to adhesions following the prior caesarean section. The following investigations were ordered: Abdominal X-rays, Abdominal ultrasound, Full blood count and Electrolytes and Urea/ Creatinine. She was placed on intravenous fluids and antibiotics. Abdominal Ultrasound showed dilated bowel loops. Abdominal X-rays showed distended bowel loops with air-fluid levels. Electrolytes and urea/ creatinine showed elevated Calcium (Ca++= 3.4mmol/L), elevated Sodium, Chloride, Urea and Creatinine levels. She was reviewed by the Medical team and a diagnosis of renal insufficiency was made (GFR- not stated). The plan was for an exploratory laparatomy once the electrolyte derangements have been corrected. On the 5th day of admission, while awaiting surgery, the patient was noticed to have suddenly stopped breathing. She was certified dead shortly after. PROVISIONAL ANATOMIC SUMMARY Fibrous adhesions between the ileum and the sigmoid colon. Shocked kidneys