| Session: Ph- chronic myeloproliferative disease |
| Case number: 078 |
Submitter(s): Carla S. Wilson, Edward N. Libby.
Clinical history
79-year-old male with persistent eosinophilia, thrombophlebitis in July 2006, and lifelong history of asthma (stable). Oxygen saturation of 94%. Asymptomatic with no hepatosplenomegaly or lymphadenopathy. Normal creatinine and liver function tests. Elevated serum IgE of 239.0 IU/ml [0-87] in March 2005.
CBCs from 7/8/03 to 1/19/07: WBC: 9.3 to 15.0 K/ul (current 11.3); RBC: 3.83 to 4.41 M/uL (current 4.41); Plt: 220 to 315 K/uL (current 315)
Differential count: 22% neut, 18% lymphs, 5% mono, 54% eosin, 1% baso, Absolute counts from 1/4/05 to 1/19/07: Eos #: 3.8 to 8.1 K/uL (current 6.1);
Neut #: 2.5 to 6.7 K/uL (current 2.5)
Medications: coumadin, Prilosec, Flomax, Plendil, albuterol (prn), Flovent (prn).
Details of gross/microscopic pathology:
Peripheral blood smear: normochromic normocytic red blood cells, increased eosinophils with mild atypia (cytoplasmic vacuolation, abnormal nuclear segmentation), single eosinophilic metamyelocyte, neutrophils without dysplasia, relative increase in large granular lymphocytes.[figure1][figure2][figure3]Bone marrow aspirate smear: normal M:E ratio (1.8:1), blasts (2%), increased eosinophils and precursors (20%) with mixed eosinophilic/basophilic granules, enlarged eosinophilic precursors with mild atypia; normal neutrophilic myelopoiesis and erythropoiesis, slightly increased megakaryocytes.[figure4][figure5]Bone marrow clot and trephine biopsy sections: hypercellular bone marrow (50%), cellular composition similar to aspirate smears, no clusters of blasts, hemosiderin laden macrophages, slightly increased megakaryocytes with occasional small and hyperchromatic forms.[figure6][figure7][figure8][figure9]
Immunophenotype (flow cytometry/immunohistochemistry):
Flow cytometry of bone marrow: Myeloblasts (CD34+, dim-moderately CD45+, CD33+) comprise 0.4% of total events.
Cytogenetics:
Karyotype (bone marrow): 46, XY, t(2;17;22)(p23;q11.2;q11.2) [16]/46,X,Y[4][figure10]FISH nuch ish (ABL1, BCR) x 2(200): no evidence for the BCR/ABL fusion associated with t(9;22) in two peripheral blood specimens (2/26/05, 11/30/06)
FISH for CHIC2, FIPIL1, PDGFRA and 4p16.3 (FGFR3): no evidence of anomalies in peripheral blood (performed at Mayo clinic).
Molecular analysis:
JAK2 V617 mutation (real time PCR): not identified in bone marrow.
Interesting feature(s) of submitted case:
Well-controlled asthmatic with new onset leukocytosis and probable eosinophilia in 2003 (well documented since 2005).
Isolated peripheral blood eosinophilia with hypercellular bone marrow due to eosinophilic proliferation. Orderly eosinophil maturation with mild cytologic atypia and no increase in blasts. Mild megakaryocytic atypia with no significant dysplasia in other lineages.
Karyotypic abnormality in 16 of 20 metaphases that involves BCR (22q11.2).
Eosinophils part of the clonal process? Indolent disorder as patient remains asymptomatic.
Proposed diagnosis:
Chronic myeloproliferative disorder, most consistent with chronic eosinophilic leukemia.
Panel diagnosis:
agree with proposed diagnosis
Comments:
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