OkhMatDit, National Specialized Pediatric Hospital
Ukrainian Ministry of Public Health
Center for Pediatric Oncohematology and Bone Marrow Transplantation
ul. Chornovola 28/1, Sholudenko 10, Kiev, 01135 Ukraine
Phone/fax +38(044)2364215, phone +38(044)2360214
Consultation as of August 18, 2010-09-02
Patient: Karina V. Koryaka, b. 1998
Place of residence: Dnepropetrovsk, Ukraine
Diagnosis: myelodysplastic syndrome (according to results of needle biopsy and
trephine biopsy of bone marrow)
Recommendations
1. Repeated bone marrow aspiration procedure with analysis at laboratory of
Center for Pediatric Oncohematology for differential diagnosis between
“adult” and “childhood” types of MDS.
2. If “adult”-type MDS is confirmed, the child should undergo allogeneic BMT
from an HLA-matching unrelated donor outside Ukraine (a potential matching
donor among family members is lacking) as the only method of radical
treatment.
3. Iа “childhood”-type (hypocellular) MDS is confirmed, a course of
immunosuppressive therapy is recommended.
4. Blood components transfusions should be made through leukocyte removal
filters.
Head of Center for Pediatric Oncohematology and BMT (Ukrainian Ministry of
Public Health)
S.B. Donskaya
----------------------------Consultation as of August 31, 2010
Patient: Karina V. Koryaka, b. 1998
Place of residence: Dnepropetrovsk, Ukraine
Diagnosis: myelodysplastic syndrome, hypocellular variant
Recommended:
1. To begin immunosuppressive therapy with Cyclosporin A 5 mg/kg/day per os
daily.
2. To perform further immunosuppressive therapy (ATG + Cyclosporin A +
Methylprednisolone) at Center for Pediatric Oncohematology.
Head of Center for Pediatric Oncohematology and BMT (Ukrainian Ministry of
Public Health)
S.B. Donskaya
============================
OkhMatDit, Ukrainian Specialized Pediatric Hospital
Case summary No. 545
Patient Karina Vladimirovna Koryaka, born Jan 28, 1998
Home address: ul. Suvorova 15a-127, Dnepropetrovsk, Ukraine
Date of admission to hospital: Jul 27, 2010
Date of discharge: Aug 6, 2010
Blood group O(I), Rh(+)
No contacts with contagious patients
Diagnosis: myelodysplastic syndrome (?), viral hepatitis B
Anamnesis morbi: Patient was admitted to Pediatric Department with paleness
and hemorrhagic skin rash. In May 2010, there was an episode of
gastrointestinal dysfunction and brief episodes of low-grade fever. No CBC
control performed. At the end of June, during summer rest at the sea, there were
paleness and fatigability. CBC test on Jul 7, 2010; according to its results, the child
was taken to Department of Hematology. Admission for trephine biopsy.
CBC with diff.
WBC,
x109/L
RBC,
x1012/L
Hb,
g/L
PLT,
109/L
Jul 27
3.1
2.2
72
2
Jul 28
1.6
2.16
71
8
Jul 30
2.3
2.19
72
3
Aug 5
2.8/1.8
1.9/1.98
63/60
2/80
Aug 6
3.0
1.93
63
72
Date
ESR,
mm/h
49
eos,
%
0
bands,
%
segs,
%
lym,
%
mon,
%
1
11
81
7
0
1
23
1
/after PLT transfusion
Urinalysis
Date
Density
pH
protein,
g/L
glucose,
g/L
WBC in
HPF
RBC in
HPF
Salts
Epithel.
Jul 28
1015
7.0
abs
abs
isol
abs
abs
abs
alk
abs
abs
isol
isol
phosph
abs
Aug 5
ANA test 1.0 (negative up to 1.5)
In view of elevated transaminase levels (found for the first time), the girl
underwent a blood test for hepatitis markers.
Blood chemistry
Jul 28: total protein 75 g/L, glucose 6.1 mmol/L, alpha-amylase 52, seroglycoids 0.15,
CRP neg., ASO neg., ALT 170, AST 74, creatinine 36 mol/L, urea 4.0 mmol/L, K
4.5 mmol/L, Na 141 mmol/L, total Ca 1.14 mmol/L.
Jul 30: glucose 6.0 mmol/L, total bilirubin 15.6 mol/L, direct bilirubin 3.4 mol/L,
ALT 165, AST 74. GGT 16, LDH 226
Cl 107, thymol turbidity test 2.4 units, RhF neg.
Protein fractions: albumin=63%, globulins=37%, 1=3%, 2=10%, =4%, =20%,
albumin/globulins=1.7.
LE test negative. CIC=145 OD units
Blood coagulation test as of Jul 28: INR 1.26 (N 0.9-1.3), PT 19.9 (N 15-20), TT 17.7
(N 13-18), APTT 30.7, fibrinogen 2.24 g/L, ethanol gelation test neg.
HBsAg, anti-HCV not detected.
Anti-HBcore(IgM+IgG) E = 0.198: antibodies to viral hepatitis B core antigen
detected.
Abdominal ultrasound as of Aug 3: Liver not enlarged, anteroposterior size 115
mm, parenchyma homogeneous, echogenicity diffusely increased, vascular
markings decreased. Gallbladder anechoic, with a kink in the region of body.
Pancreas not thickened, contour well-delineated, smooth. Spleen enlarged
(118x36x68 mm), of moderate echogenicity. Kidneys in normal location, of equal
sizes (98x36 mm), without sonographically detectable structural alterations. No
abnormal fluid accumulation detected in abdominal cavity at the moment of
examination.
Thyroid gland ultrasound as of Aug 3: thyroid in normal position, enlarged. Right
lobe 5.78 ccm, left lobe 6.51 ccm. Isthmus thickened up to 5.8 mm. Sonographic
structure diffusely inhomogeneous owing to multiple hypoechoic regions.
Ophthalmologist’s consultation as of Jul 28: Anterior segments and optic media
normal. Fundus without abnormalities.
Consultation by Head of Hematology Center S.B. Donskaya: see attachment.
During preparation for trephine biopsy, the child received platelet transfusion for
replacement purpose.
For prophylactic purposes, received amoxicillin+clavulanic acid and fluconazole.
On Aug 5, 2010, trephine biopsy was performed by hematologist at an operating
room. Before the manipulation, the patient got 2 doses of apheresis platelets and 140
mL packed RBC. Manipulation was conducted without complications; three hours
later the child was transferred to Pediatric Department.
Aug 6: stable condition, normal body temperature. According to the parents’ request,
the child was discharged from hospital to get specialized in-patient treatment at place
of residence. Recommended: specialized treatment; consultation by infectious disease
specialist.
Physician
Oxana Grishchenko
Trephine biopsy as of Aug 5, 2010
Patient: Kristina Koryaka, 13 years old, female
Biopsy: primary
Clinical diagnosis: myelodysplastic syndrome (?)
Pathologist’s report No. 19546-47 dated Aug 6, 2010
Number of fragments: 1
Number of specimens: 1
Macro- and microscopic description
Trephine biopsy specimen. Bone marrow architecture disrupted, cellularity preserved,
fatty/cellular bone marrow ratio 1:1,1:2. Moderate signs of dyserythropoiesis.
Reduced number of cells of granulocytic lineage. Increased number of cells of
histiocytic (monocytic) lineage. Total number of megakaryocytes significantly
reduced; represented by mononuclear and hypolobular forms. Increased amount
of eosinophilic leukocytes and plasma cells. Small regions of fine fibrosis.
Morphological picture may correspond to myelodysplastic syndrome.
Cytogenetic testing recommended.
====================================
Diagnostic Center of Medical Academy
pl. Oktyabrskaya 4, Dnepropetrovsk, Ukraine, phone (0562)36-19-50
Patient: Karina Koryaka, aged 12, gender female
Material: blood plasma
Date of sampling: Aug 16, 2010
Physician: T.V. Mavrutenkova
PCR assay
Hepatitis B virus DNA, hepatitis G virus RNA, hepatitis D virus RNA, hepatitis C
virus RNA: none detected.
Report handed to patient on Aug 20, 2010
=============================================
Ukrainian Ministry of Public Health
OkhMatDet clinic, Center for Pediatric Oncohematology and BMT
Reference laboratory for diagnosis of oncohematologic diseases
Patient’s name: Karina Koryaka, DOB Jan 28, 1998
Specimen No. 2010-1302 (bone marrow), 2010-1303 (peripheral blood)
Diagnosis: myelodysplastic syndrome? Aplastic anemia?
WBC differential as of Jul 8, 2010
Bands 1.0%, segs 6.0%, lym 90.0%, mon 3.0%.
Bone marrow analysis as of Jul 7, 2010
Blasts 0.4%; promyelocytes 0.4%, neutrophilic myelocytes 17.4%, neutr.
metamyelocytes 14.6%, bands 12.4%, segs 3.4%, eosinophils 3.2%, lymphocytes
11.6%, monocytes 8.0%, erythroblasts 0.8%, basophilic normoblasts 4.0%,
polychromatophilic normoblasts 13.2%, orthochromic normoblasts 10.0%,
megakaryocytes 74 in specimen.
Specimen hypercellular. Reticulum (-).
Description: Specimens are hypercellular owing to hyperplasia of all
hematopoietic lineages with moderate shift of leukoerythroid ratio toward
erythron. Myeloid lineage with marked change of maturation index toward
immature forms owing to increased content of neutrophilic myelocytes and low
content of segmented neutrophils; representatives of neutrophilic lineage with
hypergranulation. Two-lineage dyshematopoiesis: dyserythropoiesis (evidence for
megaloblastic elements, fragmentation of nuclei) and dysmegakaryocytopoiesis (some
represented by hypolobulated microforms). Megakaryocytes detected in the specimen
are without signs of functioning.
In view of CBC findings (pancytopenia) and bone marrow analysis (hyperplasia of
medullary hematopoiesis with signs of multi-lineage dyshematopoiesis), we
recommend histological and cytogenetic analysis of bone marrow for exclusion of
myelodysplastic syndrome.
Physicians: Sharamok A.I., Smolenskaya T.V., Ponomarenko T.P.
Head of Laboratory: Kreminskaya E.S.
==========================
WBC differential as of Aug 25, 2010
Bands 1.0%, segs 1.0%, lym 95.0%, mon 3.0%.
Bone marrow assay as of Aug 25, 2010
Blasts 1.2%; promyelocytes 3.0%, neutrophilic myelocytes 6.6%, neutr.
metamyelocytes 1.4%, bands 0.4%, segs 0.4%, mast cells 0.2%, lymphocytes 66.4%,
monocytes 4.2%, plasma cells 3.2%, erythroblasts 1.2%, basophilic normoblasts
4.0%, polychromatophilic normoblasts 1.8%, orthochromic normoblasts 7.0%,
megakaryocytes 2 in specimen.
Cellularity: normal. Reticulum (+).
Description: Marked hypoplasia of all hematopoietic lineages. Moderate
dysmyelopoiesis (asynchrony of maturation) and dyserythropoiesis (disruption of cell
division, irregularity of cell contours); change in the maturation index of erythroid
and myeloid lineages toward immature forms. Increased content of plasma cells and
macrophages with signs of hemophagocytosis.
Physicians: Sharamok A.I., Smolenskaya T.V., Ponomarenko T.P.
Head of Laboratory: Kreminskaya E.S.
August 26, 2010
====================================