title: Bone marrow transplant-associated thrombotic microangiopathy: a case series.
abstract: Bone marrow transplant-associated thrombotic microangiopathy (BMT-TM) has been reported with widely varying frequencies and outcomes. Therefore a BMT-TM grading system (0-4) was developed based on the lactate dehydrogenase (LD) level and percentage (%) fragmented cells (FC) as follows: grade 0, normal or increases LD and FC < or = 1.2%; grade 1, normal LD and FC > or = 1.3%; grade 2, increases LD and FC = 1.3-4.8%; grade 3 increases LD and FC = 4.9-9.6%; and grade 4, increases LD and FC > or = 9.7%. Patients with grade 0 and grade 1 BMT-TM did not differ in clinical parameters. Twenty two patients had BMT-TM grade 2-4. These were observed for outcome. Seven of 10 with grade 2 BMT-TM had resolution of BMT-TM a median of 99 days (range 50-229 days) from diagnosis. This occurred spontaneously in five and following discontinuance of cyclosporine (CsA) in two. The remaining three had persistent BMT-TM at grade 2 (two patients) and grade 1 (one patient). In contrast, none with grade 3-4 BMT-TM had resolution. Seven with grades 3-4 BMT-TM underwent a variety of plasma exchange procedures; six had partial hematologic responses. Patients with grades 3-4 BMT-TM had a poorer survival (median survival = 60 days) than those with grade 2 BMT-TM where the median survival has not been reached (P = 0.018). These results indicate that BMT-TM is common following allogeneic-BMT and the outcome is dependent on the grade. Those with grade 1-2 BMT-TM generally do well.(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Western Pennsylvania Hospital, Western Pennsylvania Cancer Institute, Pittsburgh, USA.
authors.forename: Z R
authors.initials: ZR
authors.lastname: Zeigler
authors.forename: R K
authors.initials: RK
authors.lastname: Shadduck
authors.forename: J
authors.initials: J
authors.lastname: Nemunaitis
authors.forename: D F
authors.initials: DF
authors.lastname: Andrews
authors.forename: C S
authors.initials: CS
authors.lastname: Rosenfeld
mesh_terms: D000293:Adolescent; D000328:Adult; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D016019:Survival Analysis
chemical_list: D007166:Immunosuppressive Agents