Hematology Case 61
Patient Data
Female patient 29 year old
Clinical Data
History of ecchymosis on & off one month ago.
Fever & malaise 2 days before admission.
Admitted with pancytopenia for investigations 5 days ago.
Examination: Multiple ecchymosis , subconjunctival hemorrhage, pallor. No splenomegaly & mild hepatomegaly.
WBC increased 2 days ago.
Related Laboratory Results
CBC (at admission):
- WBC: 0.6 x103 /uL (Neutrophils: 0.15 x103 /uL - Lymphocytes: 0.25x103 /uL - Eosinophils: 0.2 x103 /uL).
- HGB: 8 g/dL - MCV: 90 fL
- Platelet: 35 x103 /uL
CBC (4 days after admission):
- WBC: 12.4x103 /uL (Blast: 24% - Promyelocytes: 11% - Abnormal mononuclear cells: 20%).
- HGB: 9 g/dL
- Platelet: 40x103 /uL
PT: 20 sec. (R.R.: 11-15) - INR: 1.75 (R.R.: 0.8 - 1.2) - APTT: 51 sec.(R.R.: 25-40).
AST: 975 u/L (R.R.: 15-41) - ALT: 328 u/L (R.R.: 17-63) - ALP: 568 u/L (R.R.:30-91)
BUN: 22 mmol/L (R.R.: 2.5-6.4) - Creatinine: 169 umol/L (R.R.: 55-113).
D-Dimer: > 40000 ng/mL (R.R.: 0-500)
Provisional Diagnosis
Acute leukemia Vs aplastic anemia.
Case Picture(s) / Photo(s)
Images Comment / Findings
Peripheral Blood Smear:
Blast cells: 24% - Promyelocytes: 11% - Abnormal mononuclear cells: 20%
Severe thrombocytopenia.
Moderate normocytic normochromic anemia with schistocytes are occasionally seen (Indication for applying DIC scoring system)
Picture of Acute promyelocytic leukemia for IPT & cytogenetic workup.
Immunophenotyping by flowcytometry:
Sample : Peripheral Blood.
Positive Markers: MPO - CD13 (heterogenous) - CD33 (homogenous) - CD34 (dim) - CD64
Negative Markers: HLA-DR , TdT , CD14, CD11b, cyCD3, CD7, CD19, cyCD79a
Comment: The gated cells in PB constitute about 55% using CD45/SSC gating strategy. It expresses immunophenotyping pattern of acute promyelocytic leukemia (FAB-M3) . Cytogenetic workup for PML-RARA is indicated.
Final Diagnosis
Acute promyelocytic leukemia for PML-RARA fusion gene workup.
(Unfortunately the patient died next day of IPT reported. Cytogenetic investigations were not done.)The case was presented with DIC as per ISTH scoring system. (See below)
Additional Note
International society on thrombosis & hemostasis (ISTH) scoring system for disseminated intravascular coagulation (DIC) (CLICK HERE)
Acute promyelocytic leukaemia with PML―RARA: Clinical features : Both hypergranular and microgranular APL are frequently associated with disseminated intravascular coagulation and increased fibrinolysis. Coagulopathy is associated with significant early death rates in APL patients. (WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, International Agency for Recearch on Cancer, Revised 4th Edition, 2017, Page: 134)