Case study: Acute panmyelosis with myelofibrosis. Case 1 (Acute panmyelosis with myelofibrosis)
Clinical summary is provided by submitting physician.
Possible new acute myeloid leukemia. The marrow is performed for examination. Cytogenetic and immunophenotyping samples were also obtained.
WBC 1.1 A9/L, Neutrophils 0.14 x A9/L, Lymphocytes 0.85 x A9/L, Eosinophils 0.01 x A9/L, Monocytes 0.07 x A9/L, Myelocytes 0.01 x A9/L, Blasts 0.02 x A9/L, Hemoglobin 92 x g/L, MCV 100.7 fL, RDW 21.2, Platelets 30 x A9/L, Reticulocytes 28 x A9/L.
There is pancytopenia. A very rare blast cell is present. Occasional neutrophils are hypogranulated. Platelets include some large and giant forms as well as hypogranulation. Red cells are variably macrocytic with occasional oval macrocytes, rare hypochromic microcytes and a very, very rare teardrop poikilocyte.
Neutrophil 0.11 Late normoblasts 0.26 Plasma cells 0.00
Band cell 0.02 Intermediate normoblasts 0.03 Lymphocytes 0.32
Metamyelocyte 0.04 Early normoblasts 0.00 Monocytes 0.00
Myelocytes 0.08 Proerythroblasts 0.00 Eosinophils 0.01
Promyelocytes 0.03 Basophils 0.01
Blasts 0.09 Other cells 0.00
BONE MARROW INTERPRETATION:
The aspirate is essentially a dry tap. Dilute marrow was obtained.
M/E ratio approximately 1:1. Both erythroid and myeloid development is dysplastic. Blast cells are increased and counted at 9%. They are of intermediate size, round oval nuclei, and scant cytoplasm. Auer rods were not identified. On the touch preparations, numerous megakaryocytes are present. These are frequently of large size with a large irregularly lobulated nucleus.
A sideroblast stain is negative for ring sideroblasts. A chloracetate esterase stain confirms myeloid differentiation. A nonspecific butyrate esterase stain is positive in a rare macrophage. Reticulin is increased +3/4 by reticulin stain.
The biopsy is hypercellular at 80 to 90%. Megakaryocytes are markedly increased with numerous large pleomorphic cells as well as many smaller atypical micromegakaryocytes. Background hematopoiesis is variably preserved with some islands of erythroid activity. There is an increase in blast cells scattered diffusely through the biopsy and occasionally forming smaller 3- or 4-cell clusters that would account for approximately 10-15% of the cells. A streaming pattern is imparted to the marrow in areas.
There is trilineage hematopoiesis present with an increase in blast cells. The blast cell increase is somewhere around 10%. The pattern of megakaryocytic hyperplasia and fibrosis in the marrow raises the differential diagnosis of acute panmyelosis with myelofibrosis, refractory anemia with excess blasts and marrow fibrosis, secondary acute leukemia, acute megakaryoblastic leukemia and chronic idiopathic myelofibrosis. The current combined findings and clinical would be most consistent with acute panmyelosis with myelofibrosis although the distinction between this and acute myelodysplastic syndrome with fibrosis is unclear.