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| Case of the Week Tuesday13/12/2005 |
Presented by Dr. Marwa Khairy..
Click here to download a power point presentation for the case.
STAFF ROUND PRESENTATION
PERSONAL HISTORY
• Shaimaa Abdel Ghafaar Helmy, 20y old.
• She ' s housewife, born and living in Giza .
• She ' s married for 3 years with no offsprings.
• No history of abortion or intrauterine fetal death ( 1ry sterility ) .
• Menstrual history: menarche at 16y, with regular cycles ( 4days/ 28 days ), no history of contraception.
• No special habits of medical importance.
• History of contact with canal water, with no antischistosomal ttt received.
COMPLAINT
Upper abdominal pain
HISTORY OF PRESENT ILLNESS
• The condition started 1 year ago by bilateral hypochondrial pain of gradual onset, progressive course, dull aching in nature, with no precipitating or relieving factors.
The condition is associated with:
• Headache, dizziness, dyspnea, easy fatigability on ordinary effort.
• Recurrent attacks of bleeding per gums and epistaxis.
• Anorexia.
• Loss of weight: about 7kg through the last year.
NO :
• Manifestations of chronic liver diseases
• GIT symptoms
• Fever or rigors
• Bonyaches
• Arthralgia
• Dermatological manifestations
• Perception of body masses
• The patient sought medical advice and was told to have anaemia and liver disease.
• She received ttt in the form of liver support and iron supplements with no improvement.
PAST HISTORY
• No history of surgical operations.
• No history of blood transfusion.
• No history of diabetes or hypertension.
• No history of drug intake.
FAMILIY HISTORY
No similar condition
Negative consanguinity
GENERAL EXAMINATION
• Patient is conscious, cooperative, lying comfortably in bed.
• Pulse: 90/min
• Blood pressure: 120/80
• Temperature: 37 ° C all through hospital stay.
HEAD AND NECK
• Pallor
• No jaundice or cyanosis
• No palpable lymph nodes
• Thyroid gland is not felt.
• Parotid gland is not felt.
• Trachea is central
• Neck veins are not congested
UPPER LIMBS
• Pallor of the palmar creases
• No clubbing
• No cyanosis
• No palmar erythema
• No spider naevi
• No flapping tremors
• No ecchymotic patches
• Multiple scars at the sites of cauterized warts on the dorsum of the left hand.
LOWER LIMBS
• No oedema
ABDOMINAL EXAMINATION
• Normal shape and contour
• Subcostal angle is not widened
• Divarication of recti
• Umbilicus: shifted downwards, no impulses on cough
• No dilated veins, scars or pigmentations
• No hernias
LIVER :
- Upper border : 5th space mid clavicular line
- lower border :Rt lobe: 8cm in the MCL
Lt lobe: 18cm in the midline
The liver is firm, smooth surface, round border, no tenderness, no pulsations
• SPLEEN : 8cm below the costal margin
• ASCITES : not detected clinically
CVS, CHEST & NEUROLOGICAL EXAMINATION
FREE
FUNDUS & SLIT LAMP EXAMINATION
FREE
20 YEARS OLD FEMALE
HEPATOSPLENOMEGALY
PALLOR
CAUSES OF HEPATOSPLENOMEGALY
• Autoimmune diseases
• lymphoproliferative disorders
• Myeloproliferative disorders
• Storage diseases
• Infiltrative diseases: amyloidosis
• Infections: HCV, CMV, EBV, brucellosis
• Schistosomiasis
• Wilson ' s disease
• Haematological diseases: Thalassemia
• Protozoal infection: Malaria, Leishmaniasis
INVESTIGATIONS
SEPTEMBER 2005
URINALYSIS
• Proteins: nil
• Glucose: nil
• Acetone: nil
• Bile pigments: nil
• Urobilinogen: nil
• Pus cells: 1-2
• RBCs: 1-2
• Casts: nil
• Crystals : nil
• Ova: nil
CBC:
• WBCs
• Total: 2.500
• B= 0 %
• E= 2 %
• St= 1 %
• Seg= 64 %
• Lymph= 31 %
• Mon= 2 %
• Panctopenia :
- Mild normocytic normochromic anaemia,
RBCs show anisopoikilocytosis
- Moderate Leucopenia
- Mild Thrombocytopenia
• Retics : 3.8%
• Serum iron: 50 µ g/dl ( 37 - 157 )
• TIBC: 732 µ g/dl ( 250 - 380 )
• Ferritin: 40.50 µ g/ml ( 6.00 - 159.00 )
• Fecal occult blood (repeated): - ve
LIVER BIOCHEMICHAL PROFILE
• BIL-T : 0.89
• AST : 44 ( 0 - 41)
• ALT : 41 ( 0 - 41 )
• ALP : 231 ( 35 - 104 )
• GGT : 313 ( 7 - 32 )
• LDH : 221 ( 240 - 480)
• TOTAL PROTEIN : 7.8
• ALBUMIN : 4.0
• PC: 50%
ABDOMINAL ULTRASOUND
• LIVER : Enlarged in size, right lobe span 21cm in the right MCL, while the left lobe span 17cm in the midline, showing early coarse texture and smooth surface and normal hepatic veins. No focal lesions or IHBR dilatation. PV= 13mm.
Single hepatoduodenal lymph node is noted, measuring 2cm.
• GALL BLADDER : Average size and wall thickness, no stones or mud seen inside. CBD is not dilated.
• SPLEEN : Enlarged (longest axis 23cm), homogenous echopattern. No focal lesions. Splenic vein= 13.5mm in diameter
• KIDNEYS : Both are of average size, and parenchymal echogenicity, no typical calculi or back pressure changes.
• PANCREAS & midline structures are free.
• NO ASCITES .
• URINARY BLADDER, UTERUS AND ADNEXA: Showed no gross abnormalities.
• CONCLUSION :
- Hepatomegaly with early parenchymatous changes
- Splenomegaly
- Hepatoduodenal lymph node
UPPER ENDOSCOPY
• Esophagus: The mucosa of the lower end shows erosions with hyperaemic mucosa.
• Stomach: The mucosa of the fundus and the body is normal. The mucosa of the antrum shows diffuse hyperaemia.
• Pyloric ring: normal.
• Duodenum: The bulbar and post bulbar mucosa down to the second part shows hypertrophic oedematous folds with prominent villi and there is whitish patches on top.
• CONCLUSION:
Erosive oesophagitis
Antral gastritis
Moderate duodenitis
HEPATITIS VIRAL SEROMARKERS
• HBsAg : negative
• HBsAb : negative
• HBcAb - IgM : negative
- TOTAL : negative
• HCV Ab : negative
• HCV PCR (qualitative) : negative
AUTOIMMUNE PROFILE
• ANA: negative
• ASMA: negative
• AMA: negative
• LKM: negative
• pANCA: negative
• Copper in serum: 124 µ /dl
( 70 - 160 )
• Copper in 24hrs urine: 18.2 µ g/24hrs
( normal excretion <50 )
• Ceruloplasmin: 0.28 g/l
( 0.2 - 0.6 )
SIGMOIDOSCOPY & RECTAL SNIP
Sigmoidoscopy examination up to the splenic flexure was done showing normal mucosa.
Rectal snip was negative for shistosoma ova
• Antishistosoma antibodies : 1/80
LIVER BIOPSY
• Gross: Elongated brownish tissue core of needle biopsy specimen totally submitted.
• Microscopic:
Examination of the specimen revealed preserved hepatic architecture, no thickening of portal tracts and no cholestasis.
L ymphocytic infiltration of the portal tracts and hepatic parenchyma with minimal fibrotic changes .
No hepatocytes dysplasia or neoplasia.
No bile duct proliferation or dilatation.
No evidence of specific lesion or neoplasia
Conclusion:
Picture suggestive of active hepatitis.
• By Knodell ' s scoring system:
- activity index: 7
- fibrosis index: 1
• By Metavir ' s scoring system:
- A1 , F0
BONE MARROW ASPIRATE
• Hypercellular bone marrow.
• Myeloid series shows mild toxic granulation with otherwise normal morphology and maturation.
• Erythroid series shows mild erythroid hyperplasia with mild megaloblastoid changes in the form of cytoplasmic basophilia and karryohexis.
• Megakaryocytes are normal in number, some of them shows defective budding.
• BM platelets are present.
BONE MARROW BIOPSY
• Serial sections examined revealed mildly hypercellular BM with evident fat spaces, dilated blood vessels, multiple granuloma formation formed of megakaryocytes, eosinophils and plasma cells, mainly intertrabecular and some paratrabecular with background fibrosis.
• Reccurent hematopoietic tissues show normal number and morphology.
• No frank malignancy is detected .
• CONCLUSION:
Hypercelular marrow with multiple granulomata formation.
CAUSES OF BONE MARROW GRANULOMATA
• Infectious diseases: - bacterial ( TB )
- viral ( HIV, CMV )
• Autoimmune (rheumatoid arthritis, systemic lupus erythematosus )
• Miscellaneous conditions ( sarcoidosis )
• Malignant diseases ( Hodgkins diseases, metastatic carcinoma, leukaemia )
• Drug therapy ( chlorpropamide, allopurinol, phenytoin )
B2 microglobulin
• > 4000
SPLENIC ASPIRATE
• 77% small mature lymphocytes
• Occasional staff and segments are present ( 5% )
Conclusion:
Normal splenic aspirate morphology .
• Chest X- ray: free
• Tuberculin test: - ve
• HIV: - ve
• Cytomegalovirus: - Ig M: 0.971 ( up to 0.5 )
- Ig G: over 250 ( up to 15 )
• As the CBC of the patient improves; HB rise from 10.3 (8/9) to 11.3 (12/10) and the patient general condition improves,
the decision was taken to follow her up.
• The patient was on liver support during this period.
FOLLOW UP INVESTIGATIONS
NOVEMBER 2005
CBC
9/11/05
• WBCs: 2.700
( B=0, E=1, St=4, Seg=60, Lymph=25, Mono=8, Juv=2 )
• HGB: 11.4
• PLT: 125
• ESR: 1st hour: 40
2nd hour: 73
• Retcs: 2.1
12/10/05
• WBCs: 2.500
• HGB: 10.3
• PLT: 115
• ESR: 1st hour: 60
2nd hour: 95
• Retcs: 3.8
LIVER BIOCHEMICAL PROFILE
28/11/05
• BIL: 0.91
• AST: 94
• ALT: 133
• ALP: 220
• GGT: 257
• LDH: 430
• ALBUMIN: 4.0
• TOTAL PROTEIN: 7.4
• PC: 76%
8/9/05
• BIL: 0.89
• AST : 44
• ALT : 41
• ALP : 231
• GGT : 313
• LDH : 221
• ALBUMIN : 4.0
• TOTAL PROTEIN : 7.8
• PC: 50
ABDOMINAL US
• As previous ultrasonographic findings:
- Hepatomegaly with early parenchymal changes
- Splenomegaly
- Hepatoduodenal lymph node
HCV AB: - VE
AUTOIMMUNE PROFILE
November
• ANA : positive, speckeled
(1/40)
• ASMA : positive (1/40)
• Anti DNA: negative
• AMA : negative
• LKM: negative
• pANCA: positive
Sepember
• ANA : negative
• ASMA : negative
• AMA : negative
• LKM: negative
• pANCA: negative
SEROLOGY FOR CMV
November
• Ig M: 0.616
• Ig G: 49.5
September
• Ig M: 0.971
• Ig G: over 250
LIVER BIOPSY
Picture of chronic active hepatitis BUT more aggressive manner:
• Knodell ' s scoring:
- activity index = 11 ( was 7 )
- fibrosis index = 3 ( was 1 )
• Metavir ' s scoring:
- A2 F3 ( was A1 F0 )
Our staff round case presented with:
• Hepatosplenomegaly
• Pancytopenia
• Elevated GGT & ALP
• Rising transaminases
• ANA +ve
• SMA +ve
• pANCA +ve
• Bone marrow granulomata
• Chronic hepatitis
• CMV IgM persistently +ve
Thank you
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