Pure Red

Cell
Aplasia
(PRCA)

 An uncommon disorder in which

maturation arrest occurs in the formation
of erythrocytes
A type of anemiaaffecting the precursors
tored blood cellsbut not towhite blood
cells
Bone marrow ceases to produce red
blood cells

 In 1922
Kaznelson recognized that this
condition was a different entity
fromaplastic anemia, which presents
with pancytopenia

Etiology and Morphology

Autoimmune disease
Idiopathic
Thymoma
Lymphoproliferative
Viral infections: HIV, Herpes, Parvovirus
B19 (fifth disease) or Hepatitis

Congenital:
(hereditary)

Diamond-Blackfan anemia

Etiology and Morphology

Bone Marrow
severe lack of erythroid
precursors
myeloid precursors
megakaryocytic
elements
lymphocytes

Pathogenesis

Humoral
Cellular
Unhealthy marrow environment
Plasma inhibitor of erythropoiesis

Signs and Symptoms

Bone marrow
Decrease in the number of RBCs
deficient in the number of precursors of
RBCs(erythroblasts)
Levels of the hormone erythropoietin that stimulates
the bone marrow to produce RBCs are usually
elevated.
Fatigue, lethargy or listlessness, pallor(abnormal
paleness of skin) , poor appetite, congestive failure with
breathlessness, hepatomegaly, and splenomegaly

Screening

Confirmatory

BONE MARROW

PERIPHERAL BLOOD

Case Study
Pure red cell aplasia associated with
thymoma: clinical insights from a 50-year
single-institution experience
By :Carrie A. Thompson and David P. Steensma
Division of Hematology, Department of Medicine, Mayo
Clinic, Rochester, MN, USA

 Acquired pure red cell aplasia (PRCA) is a rare disorder

of erythropoiesis that can develop in association with a
thymoma.
Optimal management of this subgroup is unclear, and
there have been few series reporting long-term clinical
outcomes.
The median age at PRCA diagnosis was 65 years (range
3176 years) with no gender predilection (seven males
and six females) and median haemoglobin
concentration of 6.1 g/dl (range 4.89.1)

All but one patient underwent surgical excision of the

thymoma.
The patient who did not undergo resection had
significant medical co-morbidities that precluded
operative intervention
13 patients treated for PRCA associated with thymoma
over 50 years at our institution

 Surgical resection of the thymoma was insufficient for

normalisation of erythropoiesis in all cases
T-cell gene rearrangement studies did not routinely
demonstrate a clonal process, and ciclosporin and antithymocyte globulin were effective adjuvant treatments
However, treatment-related morbidity was high, with
frequent infectious complications