MINISTRY OF HEALTH OF UKRAINE
NATIONAL CHILDREN SPECIALIZED HOSPITAL
“OKHMATDIT”
28/1 Chornovola St., 01135, Kyiv Phone: 236-6942
Fax: 236-6165
CASE RECORD
KURUS VLADISLAV, 7 June 2000
DS: acquired aplastic anemia, highly severe
form. Partial remission after second course of immunosuppressive therapy.
The patient was admitted on 14 November 2009 in
severe condition after transfer from the Kherson Oblast
Children Hospital.
Severity was caused by clinical syndromes of hematopoietic aplasia and hepatic
lesions of unclear etiology.
Complete blood count 14 November 2009 – L
1,600/µl, Hb – 54 g/l, thrombocytes – 10,000/µl, neutrophils – 140/µl. In
biochemical analysis ALT level was 1200 mmol/l (30 N).
The patient was examined for viral hepatitis –
hepatitis B and C markers (IgM, IgG, PCR) are negative, positive IgG to HAV are
identified.
From 14 November – commencement of
hepatoprotector therapy: Heptral, Essenciale; course of intravenous immunoglobulin
at a dose of 1 g/kg. Fast normalization of ALT was noted. On 22 November 2009
ALT was 80 µmol/l (2 N).
After hepatoprotector therapy course and
stabilization of clinical status immunosuppressive therapy was started, in
particular, the course of antithymocyte globulin (ATG) from 24 to 21 October
2009 and Sandimmune from 30 October until now.
Follow-up myelogram dd. 15 February 2010 (day
112 from start of the therapy) – with underlying moderate cellularity aplasia
of megakaryocytic and erythrocytic cell lineages, high content of monocytes and
plasmatic cells are persistent. The child still had transfusion dependence
(administration of donot thromboconcentrate once weekly, erythromass – once
each two weeks), neutrophil level more than 500/µl at daily administration of Granocyte.
The patient was assigned to a group with bad
response so it was decided to conduct the second course of immunosuppressive
therapy.
The Course IST N 2 (thymoglobulin) was taken
from 27 February through 4 march 2010.
From 20 March 2010 in connection with increase
in neutrophil level above 2000/µl Granocyte administration was changed to each
other day regime. On 1 August 2010 neutrophil level was more than 500/µl. On 1
August 2010 no colony-stimulating factors were administered.
Follow-up myelogram dd. 30 July 2010 – with
underlying normal cellularity, erythron expansion was noted, dispnoe was not
identified, 12 megakaryocytes in the preparation, blasts 1.6%.
In September-October the patient did not
require replacement therapy with blood preparations, Hb 80 g/l, thrombocytes
28,000/µl.
Therapy characteristics: from 14 October 2010
during 3 weeks the patient received Cymeven therapy intravenously in connection
with acute cytomegalovirus infection.
During 5 weeks (April, May) the patient
received Despheral in connection with high ferritin level up to 3,400 ng/ml.
From 20 November 2010 progression of peripheral
cytopenia was noted. Neutrophil level was below 500/µl, Hb decrease to 62 g/l,
decrease in thrombocyte level to 6,000/ µl. On 30 November thromboconcentrate
trasfusion was taken. 2 December 2010 ferritin level was 1040 ng/ml.
Positive IgM and IgG to CMV were noted which
was assessed as cytomegalovirus infection. From 30 November until now the
patient received Cymeven therapy.
2 November 2010 follow-up myelogram was
performed according to which findings a decrease in cellularity, increase in
plasmatic cells percent, absence of megakaryocytes, inhibition of myeloid
lineage without impaired maturation were noted.
Considering progressing peripheral cytopenia
and negative dynamics of hematopoiesis in the patient the condition was
assesses as absence of remission o aplastic anemia.
Considering duration of disease and absence of
stable remission with transfusion dependence the patient required allogenic
bone marrow transplantation.
In connection with lack of sibling in the
family transplantation is possible from alternative donor. At present such type
of transplantation is impossible in Ukraine.
Allogenic bone marrow transplantation from non-relative
compatible donor is indicated for this patient.
The patient continues taking
Cyclosporin A at a dose of 200 mg daily.
Donskaia S.B.,
Chief of the Center
for Children Oncohematology and Bone Marrow Transplantation,
[Signature]
Kubalia N.A.,
Chief of
Oncohematology Department
[Signature]
Seal: [National
Children Specialized Hospital “Okhmatdit”, Ministry of Health of Ukraine]
6 December 2010
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