INTERESTING CASE OF ANEMIA

DR.JAYAKUMAR S A

PROF.DR.A.GOWRISHANKAR’S UNIT

• Mr. Velu;

• 42/m;

• Korukkupet-

• Manual labourer

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;

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;

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

• General examination :

conscious

oriented

afebrile

pallor +

no icterus/ cyanosis

clubbing

pedal edema

lymphadenopathy

sternal tenderness

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 ;

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

Provisional diagnosis:

Anemia for evaluation

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

• CXR report :

right mid- lower zone homogenous opacity

silhoutte sign positive

s/o mass lesion

? Bronchogenic carcinoma

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 ;

• CARDIOTHORACIC SURGEON OPINION ;

planned for thymectomy ;

transfer the patient to CTS ward ;

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; :

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 failure anemia of endocrine failure- chronic inflammation anemia 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

BONE MARROW ASPIRATION :

Normocellular ;

Erythroid series markedly

diminished;

Megakaryocytes

adequate;

Myelocytes ,lymphocytes

–normal

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

Final diagnosis

• MEDIASTINAL MASS s/o THYMOMA

• ANEMIA

• PURE RED CELL APLASIA

• 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

• Patient taken up up for surgery;

• Thymectomy done and sent for biopsy;

• BIOPSY :

BENIGN THYMOMA

TYPE AB ( MIXED)

CLINICAL STAGE 1

PURE RED CELL APLASIA:

- Triad of

“ n o r mo c h r o mic n o r mo c y t ic a n e mia ,

r e t ic u l o c y t o p e n ia < 1 % a l mo s t c o mp l e t e

a b s e n c e o f e r y t h r o b l a s t s f r o m ma r r o w “

- Kaznelson first established in 1922 as a separate

entity from aplastic anemia

Pathogenesis :

• Humoral

• Cellular

• Unhealthy marrow environment

• Plasma inhibitor of erythropoiesis

• 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

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

• 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

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

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 ;

“ 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

“Thymoma Associated With Pure Red-Cell Aplasia: Clinical Features and Prognosis “

Tomohiro Murakawa, MD, Jun Nakajima, MD, Hajime Sato, MD1, Makoto Tanaka, MD, Shinichi Takamoto, MD, Masashi Fukayama, MD2 Department of Cardiothoracic Surgery

1 De p a r t me n t o f P u b l ic He a l t h 2 De p a r t me n t o f P a t h o l o g y F a c u l t y o f M e d ic in e Un iv e r s it y o f T o k y o T o k y o , J a p a n T o mo h ir o M u r a k a wa , M D T e l : 8 1 3 5 8 0 0 8 6 5 4 F a x : 8 1 3 5 6 8 4 3 9 8 9 e ma il : mu r a k a wa - t k y @u min .a c .j p De p a r t me n t o f Ca r d io t h o r a c ic S u r g e r y , F a c u l t y o f M e d ic in e , Un iv e r s it y o f T o k y o , 7 - 3 - 1 Ho n g o , B u n k y o - k u , T o k y o 1 1 3 - 8 6 5 5 , J a p a n .

Asian Cardiovasc Thorac Ann 2002;10:150-154

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;