| Session: Ph- chronic myeloproliferative disease |
| Case number: 200 |
Submitter(s): Mary Ann Thompson, Ashwini Yenamandra.
Clinical history
73 year old female with a history of a myeloproliferative disease first diagnosed in 2000. She has had a persistently elevated WBC and splenomegaly, and she has an undocumented history of MGUS. Her course has been indolent, not requiring treatment. Her physical exam is notable for hepatosplenomegaly. Current CBC: WBC 35.8 K/ul, Hgb 7.6 g.dL, PCV 27%, platelets 215 K/ul, MCV 104 fL, reticulocyte count 6.2%. Differential is 94% neutrophils, 1% lymphocytes, 1% monocytes, 1% eosinophils, 1% basophils, 1% metamyelocytes, 1% myelocytes. Current medications include metoprolol. She has recently been transfused, and a bone marrow biopsy is performed due to her worsening anemia.
Details of gross/microscopic pathology:
The peripheral blood smear demonstrates increased leukocytes consisting primarily of segmented neutrophils and bands. There are are immature myeloids, including rare blasts. Rare basophils are noted. The bone marrow aspirate smear demonstrates trilineage hematopoiesis with no dysplasia. There is a predominance of segmented neutrophils and bands, and 3% blasts. H&E sections of the B5 fixed bone marrow biopsy demonstrate a markedly hypercellular marrow with granulocytic hyperplasia and few erythroid islands. There are multiple foci of immature myeloids, and there is mild megakaryocytic clustering. A reticulin stain demonstrates mild reticulin fibrosis.
Immunophenotype (flow cytometry/immunohistochemistry):
Flow cytometry of the bone marrow aspirate demonstrates that less than 1% of cells are CD33+ blasts by CD45 vs. side scatter gating.
Immunohistochemistry performed with antibodies to kappa and lambda light chain demonstrates a small population of polytypic plasma cells in the bone marrow biopsy.
Cytogenetics:
Karyotype analysis of bone marrow aspirates from 2000 and 2006 demonstrated a normal female karyotype. FISH performed on paraffin sections of the 2000 bone marrow particle with a BCR/ABL translocation probe demonstrated normal BCR and ABL signals in 200 nuclei. FISH performed on the 2006 bone marrow aspirate with DNA probes specific for chromosomes 5,7,8,11,and 20 demonstrated a normal signal pattern in each of 200 cells.
Molecular analysis:
None performed.
Interesting feature(s) of submitted case:
1. This case conforms to the WHO diagnostic criteria for chronic neutrophilic leukemia, with a prolonged peripheral blood leukocytosis of greater than 25 K/ul comprised of >80% segmented neutrophils and bands since 2000. The patient has hepatosplenomegaly, and has no cause of physiologic neutrophilia and no evidence for another myeloproliferative disease or myelodysplasia. The patient is Ph- by karyotype and by FISH for the BCR-ABL translocation.
2. The WHO book states that up to 20% of documented cases of chronic neutrophilic leukemia are associated with MGUS or multiple myeloma. This patient has an undocumented history of MGUS.
Proposed diagnosis:
Chronic neutrophilic leukemia.
Panel diagnosis:
Ph- chronic myeloproliferative disease, unclassifiable
Comments:
Images:
 |
Figure 1 |
 |
Figure 2 |
 |
Figure 3 |
 |
Figure 4 |
 |
Figure 5 |
 |
Figure 6 |
 |
Figure 7 |
 |
Figure 8 |
 |
Figure 9 |
 |
Figure 10 |
 |
Figure 11 |
 |
Figure 12 |
 |
Figure 13 |
 |
Figure 14 |
Back to Top
Back to Cases by Session
Back to Cases by Contact Submitter