| Session: Ph- chronic myeloproliferative disease |
| Case number: 174 |
Submitter(s): Yaneth R. Orduz.
Clinical history
This case is a 16 years old boy who consulted at the beginning of 2005 because of persistent leukocytosis and neutrofilia.
CH: WBC:37000 N: 32000 Hb: 12 Platelet count: 175000 Uric ac.: 11 DHL: 868
The diagnosis at that time made on a bone marrow biopsy was Myeloproliferative/Myelodysplasic disorder, unclassificable (made in another Institution by an Hematopathologist)
He had splenomegaly by images and the course of his disease progressed in 2006 increasing his anemia (Hb: 8) and having more physical complaints as fatigue, persistent headache, astenia and adinamia. He also had skin lesions that were biopsed.
May/2006 WBC: 31000 N: 82% most of them hypolobated (pseudo-Pelger-Huet) Hb: 11.3 Platelet count: 105000 DHL: 988
Pre-trasplant bone marrow studies were done in July 2006 but any of his two sisters were HLA compatible.
He recieved hydroxiurea but as he lived in another city diferent from Bogota, He died because his disease but we do not have more information about it.
Details of gross/microscopic pathology:
May/2006 We recieved a crest-iliac bone marrow biopsy that was fixed in formalin.
The cellularity was 99% with marked predominance of granulopoiesis representated mainly by its precursors (myelocytes and metamyelocytes). Erythropoiesis was decreased. Reticulinic fibers were not increased.
He also had some skin lesions that were investigated in order to confirm skin infiltration by his leukemia. CD34, MPO and CD68 (PGM1) were done on a formalin fixed biopsy but they did not confirm infiltration. Another skin biopsy was done and informed as panniculitis without leukemic skin infiltration in another institution.
Immunophenotype (flow cytometry/immunohistochemistry):
There was a predominance of granulopoiesis but without increase of blasts.
Cytogenetics:
May/ 2005: 47 XY, trisomy 8
May/2006: 48 XY, trisomy 8 and trisomy 21.
Molecular analysis:
BCR/ABL oncogen PCR : negative (was done in another Lab).
Interesting feature(s) of submitted case:
This case is very challenge because it did not fit well any other Chronic Myeloproliferative or Myelodysplastic/Myeloproliferative disorder based on their criteria, as Chronic Myelomonocytic Leukemia, even the Juvenil one.
It fit most of Chronic neutrophilic leukemia criteria so that diagnosis was suggested even of the rarity of this disease.
Proposed diagnosis:
Chronic neutrophilic leukemia.
Panel diagnosis:
MDS/MPD, unclassifiable
Comments:
Panel comment: Original material along with the bone marrow differential count has to be reviewed for definitive diagnosis.
Images:
 |
Figure 1 |
 |
Figure 2 |
 |
Figure 3 |
 |
Figure 4 |
 |
Figure 5 |
 |
Figure 6 |
 |
Figure 7 |
 |
Figure 8 |
 |
Figure 9 |
 |
Figure 10 |
Back to Top
Back to Cases by Session
Back to Cases by Contact Submitter