Contributed by Hazim Najjar, MB, BS, MD
Published on line in September 2000
PATIENT HISTORY:
A 68 years old white female known case of COPD, IHD and severe scoliosis, transferred to UPMC because of worsening anemia, thrombocytopenia, renal insufficiency and markedly elevated calcium and uric acid.
SELECTED LAB RESULTS:
CBC Hgb 7 Hct 20.7 WBC 7.5 RBC 2.12 MCV 97.4 MCH 33.2 RDW 16.4 Plt 44 |
Selected Electrolytes Ca 12.7 Phos 6.6 Mg 1.1 Uric Acid 23.4 Others were normal |
Renal Function BUN 67 Creatinine 4.9 Amylase 101 |
Further Tests:
Peripheral Blood:
Peripheral blood shows involvement with blastic plasma cells, anemia, thrombocytopenia and leukoerythroblastic blood picture.
Bone Marrow:
-Differential: Plasma Cells 90%
-Many of the cells counted as plasma cells appear blastic with very prominent nucleoli and variable amount of basophilic cytoplasm.
Biopsy:
-Small and fragmented but adequate.
-The marrow appear to be hypercellular and almost completely replaced by a diffuse proliferation of large cells with large nuclei having very prominent nucleoli and variable amount of basophilic cytoplasm. Normal hematopoietic elements are present but markedly decreased.
Special Stains:
-Anti-kappa: negative.
-Anti-lambda: positive with background staining.
Flow Cytometry:
-Prominent population of CD45 negative, CD56 in part positive cells with little to no CD138 positivity.
-There dose appear to be a large amount of cytoplasmic lambda positivity with possibly some weak staining.
Cytogenetics:
41-50,X,-X[9],del(1)(q32)[3],+der(1;6)(p10;q10)x2,add(2)(q37)[9],+3[3],-8[5],+9,-12[9],+13[4],add(14)(q32)[9],+14,+15[2],-16[9],+20[3],+21[8],-22[4],+mar1,+mar2[5][cp9]/46,XX[3]
Mosaic abnormal female bone marrow chromosome analysis with an apparently normal cell line and one that appears to have multiple clonal chromosome abnormalities. Most of the abnormal cells had only one X chromosome; two additional derivative chromosomes, each containing the entire long arm of chromosome 1 and the entire short arm of chromosome 6, resulting in tetrasomy 1q and 6p; an abnormal chromosome 2 with an additional chromatin segment attached to the distal long arm; monosomies 8, 12 and 16; trisomies 9 and 21; an abnormal chromosome 14 with an additional chromatin segment attached to the distal long arm (14q+); and two marker chromosomes.
Serum protein Electrophoresis:
Decreased Prealbumin, Albumin, Alpha 2 and Gamma Globulins. Monoclonal band (1.2 gm/dl) detected in Beta region.
Urine Protein Electrophoresis:
Tubular Pattern of Proteinuria. Monoclonal Free light chain (60 mg/dL) in Gamma region.
Serum Immuno-Electrophoresis with Immuno-Fixation:
Monoclonal IgD/Lambda (1.2gm/dL) detected in Beta region.
Clinical Course
-Patient developed progressive respiratory distress.
-Transferred to MICU.
-Intubated and mechanically ventilated.
-Developed worsening ARDS.
-Died after 9 days of admission.