Prepared by Porf. Dr. Ayman Yousry Unit.

Presented by Dr. Alaa Aboud .

Click here to download a power point presentation for the case.

Acute Pure Red Cell Aplasia

STAFF ROUND PRESENTATION

Personal History

•  Male patient, Mohammed Mahmoud Ali 53 years old, born in Mansoura & lived there for 10 years, now lives in Giza, works as a teacher, married with 2 offsprings the youngest is 12 years old.

•  He used to smoke 10 cig. / day for 20 years & stopped 1month ago.

•  There is history of contact with canal water and antischistosomal treatment in the form of injections.

Complaint

Cough followed by Dyspnea .

Present history

•  The condition started one month ago by cough with yellowish expectoration not related to posture or exertion associated with low grade fever reaching 38.5° C all over the day.

•  The patient sought medical advice at outpatient clinic & was told to have chest infection .

•  He received medications in the form of antibiotics with improvement .

•  One week later the patient started to develop dyspnea on ordinary effort, palpitation and easy fatigability.

•  There is no orthopnea or paroxysmal nocturnal dyspnea .

•  The condition was associated with tingling & numbness of both upper and lower limbs.

•  There was no headache, dizziness, tinnitus or blurring of vision.

•  No history of bleeding from body orifices, weight loss or perception of body masses.

•  The patient is known to be diabetic for 9 years started on diamicron and now on insulin.

Past history

•  No history of surgical operations.

•  No history of drug intake.

•  No history of previous hospital admission.

Family history: Irrelevant
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General examination

•  The patient is fully conscious, of average intelligence and lying comfortably on bed.

•  Pulse: 120/min, regular, water hammer pulse.

•  Blood pressure: 130/80 .

•  Temperature: afebrile all through the hospital stay.

•  Pallor.

•  Right preauricular & submental L.Ns: 0.5 x 0.5cm and 1x1cm respectively, firm, rounded & not tender. No other palpable lymph nodes.

•  No jaundice or cyanosis.

Abdominal examination

Inspection :

•  Epigastric pulsations.

•  Subcostal angle: acute.

•  Recti: not divaricated.

•  Umbilicus: normal site & shape with No impulse on cough.

•  No dilated veins, hernias, scars or pigmentation.

Palpation

Liver:

•  Upper border: 5th space midclavicular line.

•  Lower border : right lobe is felt 4 cms below the costal margin with smooth surface, firm consistency, rounded border and no tenderness. Left lobe is not felt.

Spleen : not felt.

No ascites detected clinically.

Cardiovascular System

•  Supersternal pulsations.

•  Epigastric transmitted pulsations.

•  Accentuated heart sounds.

•  No cardiac murmurs.

Chest examination:

Free

Neurological Examination:

Glove & Stock hypothesia.

Investigations

•  Urinalysis: normal.

•  Stool analysis: normal.

CBC

HGB : 5.4 g/dl

RBC: 1.84 millions

HCT : 15.5 %

MCV: 84.1 fL

MCH: 29 pg

MCHC: 34 g/dl

W.B.Cs 10000 /ul

BA: 0.4%

EO: 0.2%

LY:8.4%

MO:8.1%

NE:82.9%
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Conclusion

•  Marked normocytic normochromic anemia.

•  Mild polymorph nuclear Leucocytosis.

•  Absolute Lymphopenia.

•  Reticulocytic count:

0.1 % N:(0.2-2)

•  E.S.R:

First hour: 140 mm/hr

Second hour: 155 mm/hour

Liver biochemical profile

•  Bilirubin :

• Total: 0.75 mg/dl

• Direct: 0.03 mg/dl

•  AST: 28 U/L (0-37)

•  ALT: 22 U/L (0-41)

•  ALP: 97 U/L (35-104)

•  GGT: 44 U/L(8-61)

•  Total ptn: 5.9 g/dl (6.6-8.7)

•  Albumin: 3.2 g/dl ( 3.4-5.2)

Electrolytes

• Serum sodium: 140 mmol/l (132-145)

• Serum potassium: 3.4 mmol/l (3.6-5.5)

Kidney function tests

• Urea: 45mg/dl (7-50)

• Creatinine: 0.78 mg/dl (o.3-1.2)

Blood sugar

• Fasting: 143 mg/dl (60-110)

• 2 hours pp: 200 mg/dl (70-140)

•  Iron: 47 ug/dl (59 -158)

•  TIBC: 201ug/dl (274-385)

•  Ferritin: 129.80ng/ml (28-397)

•  Occult blood in stool: Negative
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Autoimmune profile:

•  ANA: negative

•  ASMA: negative

•  AMA: negative

•  Anti LKM-1: negative

•  Direct coombs: negative

•  Cold agglutinin: 1/4

•  Brucella agglutination test

Negative.

Abdominal ultra-sound

Liver: Enlarged in size with bright echopattern & regular surface. Hepatic veins are normal. No focal lesions or IHBRs dilatation. PV is not dilated.(11mm)

G.B.: is of average size and wall thickness. No stones or mud inside. CBD is not dilated.

Spleen: is of average size (11.6 cms) and homogenous echopattern. Splenic vein measures 8.3mms.

Kidneys : both are of average size, and normal parenchymal echogenicity No typical calculi or back pressure changes.

Midline structures and pancreas are free.

Prostate is mildly enlarged (volume= 25.2cm).

Conclusion:

•  Bright hepatomegaly.

•  Mild Prostatic enlargement.
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Chest x-ray

•  Clear both lung fields with increased bronchovascular markings.

•  Clear both costophrenic angles.

•  Normal cardiac shadow size.

CT Chest

Normal study

Echo-cardiography

Normal study

Bone Marrow Examination

•  Bone marrow aspirate:

•  Cellularity: normocellular

•  Myeloid:relative hyperplasia with normal morphology and maturation.

•  Erythroid: moderately depressed with moderate dyserythropoiesis.

•  Megas:are present with normal cytoplasmic granulation,nuclear lobulation and platelets budding with some dysplastic features.

Conclusion:

•  Normocellular bone marrow with selective erythroid depression.

•  Viral causes of anemia should be excluded.

•  Follow up after 2 weeks by bone marrow aspirate.

Follow up after 2 weeks:

•  Cellularity: normocellular

•  Myeloid: relative hyperplasia.

•  Erythroid: markedly depressed.

•  Megas: are present with normal cytoplasmic granulation, nuclear lobulation and platelets budding with some dysplastic features.
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Bone marrow biopsy:

•  Gross picture: A core of bone tissue.

•  Microscopic picture: B.M trabeculae separating mildly hypercellular bone marrow with areas of marrow hge, frequent dilated blood vessels (2nd degree marrow fibrosis).

•  Megakaryocytic lineage: are hyperplastic and increased in number with dysmegakaryopoesis (dysplastic morphology & topography).

•  Myeloid: seen with normal mophology.

•  Erythroid: small regenerating foci of erythroid cells are seen mainly in sinuses.

•  Occasional mature looking lymphoid elements are encountered.

Conclusion

•  Peripheral blood anemia with hypercellular marrow with monolineage dysplasia and nest of erythroid population

•  Picture suggestive of acute pure red cell aplasia.

Follow up of CBC after 1month

HGB: 8.9 g/dl

RBC: 2.87millions

HCT: 25.2 %

MCV: 87.1 fL

MCH: 31 pg

MCHC: 35 g/dl

W.B.Cs: 6400 /ul

BA: 0%

EO: 1%

LY: 38%

Staff: 5%

Seg: 56%

Reticulocytes: 2.1%

Thank you

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