A Case of Pure Red Cell Aplasia

1. INTERESTING CASE OF ANEMIA
DR.JAYAKUMAR S A
PROF.DR.A.GOWRISHANKAR’S UNIT

2. •Mr. Velu;
•42/m;
•Korukkupet-
•Manual labourer

3. c/o :-
breathlessness on exertion 6months
cough
HOPI :-
patient was apparently normal 6 months before ;
h/o breathlessness present; grade 1;
no h/o PND/orthopnea/chest pain /palpitation;
h/o dry cough ; intermittent ; no diurnal predilection;
h/o easy fatigability +
no h/o /fever /weight loss /loss of appetite ;
no h/o bleeding p/r /hemetemesis/melena;

4. No h/o cold intolerance /constipation /voice change ;
No h/o polyuria/polydipsia /polyphagia;
No h/o loose stools /vomiting/altered bowel habits;
No h/o yellowish discoloration of urine ;
No h/o abdominal distension/swelling of legs;
No h/o weakness of any limbs/sensory disturbance ;
PAST HISTORY:
not a known DM/HT/IHD/BA/ TB/EPILEPTIC;
no history of any surgeries /blood transfusions/drug intake;

5. Personal history :
married with two sons;
used to smoke two packs of beedi/d
stopped 2 yrs back;
used to consume alcohol regularly
stopped 2 years back ;
denies promiscuous behaviour
takes mixed diet ;
Family history:
insignificant

6. •General examination :
conscious
oriented
afebrile
pallor +
no icterus/ cyanosis
clubbing
pedal edema
lymphadenopathy
sternal tenderness

7. Vitals:
PR- 88/mt;
felt in all peripheral pulses equally;
BP- 110/80 mmHg;
System examination :
CVS:
ApicaI impulse – normal in position;
S 1 S 2 heard ;
haemic murmur heard in all areas ;
no thrill ;

8. RS:-
trachea in midline;
normal vesicular breath sounds heard in all
areas;no added sounds ;
ABDOMEN:
soft,
no organomegaly;
no free fluid;
CNS:
higher functions/motor,sensory,cranial nerves- normal;
no involuntary movements;
spine cranium normal

9. Provisional diagnosis:
Anemia for evaluation

10. Investigations:
CBC:-
Hb- 4.5g/dl RBS-134 mg
Tc - 4100 ; P 74 L 26; Urea-26.0mg
ESR – 24/55; Creat.- 0.9mg
Platelet -2.0 lakhs;
PCV – 20%;
MCV – 84.4
MCH -28.1
MCHC- 33.3

12. •CXR report :
right mid- lower zone homogenous opacity
silhoutte sign positive
s/o mass lesion
? Bronchogenic carcinoma

16. CT chest plain and contrast:
lobulated large soft tissue
density noted in anterior
mediastinum on right side
encasing the SVC, ascending
aorta ;
USG correlation –
thyroid seen separately
s/o thymoma ;

17. •CARDIOTHORACIC SURGEON OPINION ;
planned for thymectomy ;
transfer the patient to CTS ward ;

18. Peripheral smear :
normocytic normochromic anemia ;

Reticulocyte count : 0.5 %
serum iron: 110 ųg/dl;;( 50 – 150 )
TIBC : 300 ųg/dl; ( 300 – 360 )
Protein electrophoresis: normal
HIV: negative ;
ECG: normal
ECHO: normal ;
USG ABDOMEN:
normal ; no organomegaly
no free fluid; :

19. Approach to normocytic anemia
check reticulocyte count
normocytic anemia
increased
Is there
evidence of
hemolysis?
hemolytic
anemia
yes
Is there evidence of:
- renal failure anemia of renal failure
- endocrine failureanemia of endocrine failure
- chronic inflammationanemia of chronic disease
normal or decreased
recent
bleed
no
If not, then consider
a primary marrow problem (MDS, MM, infiltration…)
bone marrow investigation
(Pure red cell aplasia

20. BONE MARROW
ASPIRATION :
Normocellular ;
Erythroid series markedly
diminished;
Megakaryocytes
adequate;
Myelocytes ,lymphocytes
–normal

22. Haematologist opinion
CH.NO.4780/10
•Bone marrow picture
suggestive of
‘ PURE RED CELL APLASIA ‘
ADV:
inj. Dexamethasone 8 mg bd
foll.by
T.prednisolone 1mg/kg daily
review after one month

23. Final diagnosis
•MEDIASTINAL MASS s/o THYMOMA
•ANEMIA
•PURE RED CELL APLASIA

24. •Before transferring the patient to CTS
5 units of blood transfused for the patient ;
Repeat CBC values :
Hb -8.2 g /dl ;
TC – 7100 P64 L34 E2
ESR -14/30
PCV- 28%;
MCV-81.0 fl
MCHC-32g/dl
MCH-28 pg

25. •Patient taken up up for
surgery;
•Thymectomy done and sent
for biopsy;
•BIOPSY :
BENIGN THYMOMA
TYPE AB ( MIXED)
CLINICAL STAGE 1

27. PURE RED CELL APLASIA:
- Triad of
“ nor mochr omic
nor mocyt ic anemia ,
r et icul ocyt openia < 1 %
al mos t compl et e
abs ence of er yt hr obl as t s
f r om mar r ow “
-Kaznelson first established in 1922 as a
separate
entity from aplastic anemia

28. Pathogenesis :
•Humoral
•Cellular
•Unhealthy marrow
environment
•Plasma inhibitor of
erythropoiesis

29. •Congenital:
–inherited –Diamond Blackfan
–non inherited –Pearson’s
•Acquired:
–Primary
–Secondary
•hemat.malignancies
•Thymoma
•solid tumors
•Infections- parvovirus B19
•Drugs
•Pregnancy
•severe renal failure
•nutritional deficiences

30. Drugs causing PRCA :
A – allopurinol ,alpha methyl dopa, azathioprine
C- carbamazepine ,chloramphenicol ,cotrimoxazole
E- estrogens,erythropoietin
H- halothane
I – INH
P- penicillins ,procainamide ,D-penicillamine
R –rifampicin
S – sodium valproate
T – tacrolimus

31. •Incidence of thymoma in PRCA patients– 9 %;
•Incidence of PRCA in patients with thymoma -4 %;
Clinical features of PRCA :
Signs & symptoms of anemia ;
hepatosplenomegaly & absent
lymphadenopathy
LABORATORY FEATURES:
hallmark - ‘absence of erythroblasts from otherwise
normal marrow ‘
others: iron stores elevated ;
erythropoietin elevated

32. Treatment of PRCA :
1. Stop any drugs suspicious of causing PRCA;
2. IVIg for Human B19 parvovirus associated PRCA;
3. Folate and B12 supplementation ;
4. In case of thymoma don’t start immunosuppressives
before doing thymectomy;
5. Prednisone: 1- 2 mg/kg /d till remission ;
not longer than 12 weeks ;
if PCV reaches 35 % start tapering
assess response by weekly Hb& retic count

33. Other treatment options:
cyclophosphamide/ azathioprine
cyclosporine
ATG
rituximab
plasmapheresis
splenectomy
maintenance blood transfusions
Prognosis :
spontaneous remission – 5 to 10 % ;
with immunosuppressives – 68 %;
relapse within 2 years – 80%;
median survival – 14 yrs ;

34. “ Successful Treatment of a Patient with a Thymoma
and Pure Red-Cell Aplasia with Octreotide and
Prednisone”
Giovannella Palmieri, M.D., Secondo Lastoria, M.D., Annamaria Colao, M.D., Ph.D.,
Emilia Vergara, M.D., Paola Varrella, M.D., Edoardo Biondi, M.D., Carmine Selleri,
M.D., Lucio Catalano, M.D., Gaetano Lombardi, M.D., Angelo Raffaele Bianco,
M.D., and Marco Salvatore, M.D.
N Engl J Med 1997; 336:263-265J

35. “Thymoma Associated With Pure Red-Cell Aplasia: Clinical
Features and Prognosis “
Tomohiro Murakawa, MD, Jun Nakajima, MD, Hajime Sato, MD
1
,
Makoto Tanaka, MD, Shinichi Takamoto, MD, Masashi Fukayama,
MD
2
Department of Cardiothoracic Surgery
1
De par t me nt of Publ ic He al t h
2
De par t me nt of Pat hol og y F ac ul t y of Me d ic ine
Univ e r s it y of Tok y o Tok y o, J apan Tomohir o
Mur ak awa, MD Te l : 8 1 3 5 8 0 0 8 6 5 4 F ax : 8 1 3 5 6 8 4
3 9 8 9 e mail : mur ak awa- t k y @umin.ac .j p De par t me nt
of Car diot hor ac ic S ur g e r y , F ac ul t y of Me d ic ine ,
Univ e r s it y of Tok y o, 7 - 3 - 1 Hong o, Bunk y o- k u,
Tok y o 1 1 3 - 8 6 5 5 , J apan.

Asian Cardiovasc Thorac Ann 2002;10:150-154

37. CARRY HOME POINTS
PRCA is a rare cause of anemia ;bone marrow examination
is diagnostic ;
It is a curable condition ;
Steroids ,blood transfusions are the main stay of treatment ;
If thymoma is associated do thymectomy first before
immunosuppression ;
IvIg used for B19 parvovirus ass. PRCA;
It can be precede the development of leukemia ,MDS etc,
always follow up should be insisted ;
Erythropoietin used in CKD can also cause PRCA;
Darbopoietin has less incidence of PRCA;