Medical Diagnosis

Vilnius, April 06, 2001
Patient: Zaneta Zmoginaite Date of birth: 1981 03 12

Diagnosis: Acute lyniphoblastic leukemia, pre-B, 1" bone marrow and CNS relapse 

The ALL pre-B. CALLA+ was diagnosed in September, 1998. At diagnosis WBC 8,39x10⁹/1, RBC 2,15x10¹²/1, HGB 75,4 glI, PLT 81,8x10⁹/1, blasts 78%; bone marrow biopsy: cariocytes 35Ox10⁹/1, blasts 93%, all myeloperoxidase negative; peripheral blood blasts by flowcytometry: CD45+^dim, CD 19+, CD 10-, CD34+, CD22+^dim, CD13+^dim, HLA-DR+.

Induction (Sept-Oct, 1998): vincristine, daunorubicine, prednisone, L asparaginase, 6-MP, cytarabine, and cyclophosphamide. 1^st CR was obtained within 4 weeks. 

Consolidation consisted of high dose methotrexate, ifosfamide, vincristine, cytarabine, etoposide, dexamethasone alternated with high dose methotrexate, cyclophosphamide, vincristine, doxorubicin, dexamethasone for a total of 7 courses. 

Intrethecal prophylaxis: monthly methotrexate, cytarabine, dexamethasone. 

The patient was in continuous CR during consolidation therapy. Unfortunately, against medical advice she refused any further treatment in July, 1999. 

Her leukemia relapse was evident in Feb, 2000. The patient once again declined treatment but eventually was admitted to hospital on March 10, 2000. 

On admission massive hepatosplenomegaly as well as lymphadenopathy was evident. CBC: WBC 63x10⁹/1, blasts 92%, myelocytes 1%, segm 1%, lymph 6%, Hb 59 g/1, PLT 9x10⁹/1. LP: 1 blast/3µl. 

The Hyper-CVAD, HDMtx-HDAraC protocol was chosen as salvage treatment. Hyper-CVAD with addition of L asparaginase was started on March 10, 2000. There was a fast disappearance of peripheral blast cells as well as organomegaly. The neuroleukemia was treated with intrathecal mtx, AraC and dexamethasone. The post cytostatic aplastic period was protracted with bacterial and suspected fungal complications necessitating G-CSF treatment in addition to antibiotics and antifungal agents. BM biopsy was performed during pancytopenia (WBC < 0. 1 x 10⁹/1, Hb and PLT maintained by transfusions) and on G-CSF treatment on davs 19^th and 21^th post cytostatic treatment showing very hypoplastic bone marrow with 10% blast cells. The decision whether the remission of leukemia had been obtained was postponed because of some doubts regarding interpretation of BM biopsy results (no remission vs. regenerating marrow). The G-SCF was continued for another 6 days. The WBC count increased to 0.4x10⁹/1, ANC to 0.2x10⁹/1. The patient is still transfusion dependent, there is slight hepatomegaly. Otherwise, the patient has suspected fungal infection in the lungs, but is afebrile and in stable medical condition. 

She will be discharged from the hospital for a few days for psychological reasons, antimicrobial and antifungal treatment being continued. The readmission is scheduled for April 10, 2000 for BM examination and further treatment with HDMtx-HDAraC.

We do appreciate that even if the 2^nd CR is obtained, the long-term survival is highly unlikely without an alloBMT. Unfortunately, our patient has no related donor, neither is Lithuania participating in any unrelated donor program.

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