An Unusual Case Of Myelodysplastic Syndrome With Prolonged Clonal Stability, Indolent Clinical Course Over A Decade, And Spontaneous Regression Of Acute Myeloid Leukemia In The Terminal Phase
Sonneck, K., Mannhalter, C., Krauth, M. T., Sperr, W. R., Schwarzinger, I., Fonatsch, C., Haas, O., Geissler, K., & Valent, P. (2005). An unusual case of myelodysplastic syndrome with prolonged clonal stability, indolent clinical course over a decade, and spontaneous regression of acute myeloid leukemia in the terminal phase. European journal of haematology, 75(1), 73–77. https://doi.org/10.1111/j.1600-0609.2005.00423.x
View Original Source →Abstract
An unusual case of secondary acute myeloid leukemia (AML) with indolent clinical course is described. The patient, a 67-yr-old female, had first been diagnosed to suffer from low-risk myelodysplastic syndrome, subtype refractory anemia with ringed sideroblasts, in 1992. In 2001, transformation to secondary AML with an increase in bone marrow blasts (>20%) and thrombocytopenia, was found. The patient did not require cytoreductive drugs. Rather, during the following months, spontaneous improvement of peripheral blood cells with normalization of platelets and decrease in the red cell transfusion frequency, were noted. In October 2002, she even became transfusion independent. However, the bone marrow still showed AML with >20% blasts. These blast cells exhibited a monoclonal pattern in the human androgen receptor (HUMARA) assay. However, no chromosomal defects occurred during a total observation period of 14 yr. We hypothesize that clonal stability may have contributed to the indolent course of the disease in this patient. The exact mechanisms underlying clinical and genetic stability remain unknown, however.
Case Details
Disease Location
Bone marrowithblood
Personal Characteristics
56 -year-old female
Clinical Characteristics
Patient was referred in october 1990 because of mild macrocytic anemia in 1992, marrow exam revealed dysplastic features in myeloid cells and an increase in ringed sideroblasts but no increase in myeloblasts diagnosis of low-risk mds subtype refractory anemia with ringed sideroblasts was made during the next 9 years, hgb levels decreased slightly but in 1999 hgb dropped significantly september 1999, erythropoietin therapy was started and was given continuously until december 2000 and resulted in a slight increase of hgb but in dec 2000 hgb decreased again between dec 2000 and july 2002, erythrocyte transfusions were administered to keep hgb above 8g/dl in september 2001, neutropenia and thrombocytopenia occurred, marrow exam revealed 24% blasts cells consistent with secondary acute myeloid leukemia at age 67, diagnosis of acute myeloid leukemia had been established in april 2003, blast cells were detected in the differential blood count and she became tranfusion-dependent again in may 2003, re-exam of the marrow showed persistence of acute myeloid leukemia during the next few weeks, peripheral blasts cells further increased and required red cell concentrates in october 2003, 20% blasts were recorded in differential counts during the next few months, clinical situation deteriorated and the patient died from progressive acute myeloid leukemia with multiorgan failure in april 2004 marrow acute myeloid leukemia blasts examined in 2001 and 2003 were found to be medium sized with a basophilic cytoplasm and a prominent nucleus containing one or two nucleoli histology showed a hypercellular marrow with replacement of the normal marrow space by blast cells flow cytometry found blast cells expressed mpo, CD13, 33, 34, and HLA-dr, surface expression of CD14 and 34 were confirmed by ihc humara confirmed the presence of a monoclonal population of leukemic cells
Remission Characteristics
After acute myeloid leukemia diagnosis at age 67, the blood picture improved during the next few months, platelets and leukocytes increased back to normal and frequency of erythrocyte transfusions decreased in oct 2002 she was tranfusion independent which lasted until april 2003 in july 2003, a remarkable decrease in blast cells was recorded
Treatment & Mechanisms
Proposed Remission Mechanisms
The capacity for marrow to produce enough erythroid cells to escape transfusion dependence maybe the blood transfusions
Clinical Treatment
Erythropoietin therapy, red blood cell transfusions
Non-Clinical Treatment
None reported