While waiting for BM diagnosis patient was managed with packed cells, FFP,Platelet concentrate,antibiotics
BMA done on 29/7/11
Lymphoblast & myeloblast
Marrow contains 90% blast cells comprising of both lymphoblast and myeloblast
BILINEAL OR BIPHENOTYPIC ACUTE LEUKEMIA with Deep vein thrombosis
BIPHENOTYPIC ACUTE LEUKEMIA(BAL)
Biphenotypic acute leukaemia is an uncommon type of leukemia.
The precise incidence is uncertain, it is likely to account for approximately 5% of all acute leukemias.
BAL can be de novo or secondary to previous cytotoxic therapy.
It has been included in the WHO classification of haemopoietic malignancies as acute leukaemia of ambiguous lineage
WHO classification of hematological malignancies
Chronic myeloproliferative disorders
Leukemia of myeloid origin
Leukemia of lymphoid origin
Leukemia of ambiguous origin
Acute leukemias of ambiguous lineage include
1.Undifferentiated acute leukemia
Leukemic cells that can not be classified as
either myeloid or lymphoid cells
2. Acute leukemias of mixed phenotype.
Mixed lineage or biphenotypic acute leukemia
where both types of cells are present.
EGIL scoring for biphenotypic acute leukaemia. The score for each lineage is calculated and if that lineage score > 2, that lineage is present. EGIL-European group for immunological classification of leukemia POINTS T CELL LINEAGE B CELL LINEAGE MYELOID LINEAGE 2 POINTS CD 79 CD3 TCR MPO 1 POINTS cCd22 CD10 CD19 CD20 CD2 CD5 CD8 CD10 CD13 CD33 CDw65 CD117 0.5 POINTS Tdt CD24 Tdt Cd17 cd1A CD15 CD15 CD24
BAL has proven to be very difficult to treat with many cases being resistant to induction chemotherapy and those that enter remission have a high risk of relapse.
There is no agreement on how the disease should be treated.
The majority of patients receive treatment according to the morphology of the blasts, with either AML or ALL induction.
If patients enter complete remission, consideration should be given to consolidation with stem cell transplantation.
When immunophenotyping is not possible steroid trial should be started
Prognostic factor for CR
Prognostic factor for survival
achievement of CR
The prognosis of biphenotypic acute leukemia patients is poor when compared with de novo acute myeloid leukemia or acute lymphoblastic leukemia.
Biphenotypic acute leukemia patients have abnormal karyotype, extramedullary infiltration, relapse, and resistance to therapy after relapse
DVT IN ACUTE LEUKEMIA
Studies have demonstrated that the risk of VTE associated with the hematological malignancies acute leukemia, lymphoma, and multiple myeloma is considerable.
Contributing factors include hypercoagulablefactors,Hyperleukocytosis,
antineoplastic therapies such as high dose corticosteroids, L-asparaginase, and new immunomodulatory agents; central venous catheters; and hematopoietic growth factors .
Primary and secondary pharmacological prophylaxis can be problematic in these patients who are often thrombocytopenic. Strategies to prevent VTE, especially upper extremity catheter-associated thrombosis need to be developed.
The incidence of VTE at diagnosis was
1.2 % in ALL,
3.2% in APL and
2.5 % in non-M3 AML.
Thromboembolism is a well recognized complication of hematologic malignancy. The pathogenesis of the cancer-related prothrombotic state are
1. activation of blood coagulation via procoagulant substances,
2.impairment of fibrinolytic pathways,
3.alterations of endothelium toward a thrombogenic state
Journal of Thrombosis and Haemostasis, 3: 1985–1992
The classic "gold standard" is contrast venography. Although very accurate, this method requires radiologic facilities and expertise and is invasive and sometimes uncomfortable for the patient.
Ultrasonography, with noncompressibility of the vein as the sole criterion, has largely replaced contrast venography
investigation is limited to the femoral vein in the groin and the popliteal vein in the popliteal fossa
The combination of the assessment of clinical probability and the measurement of the D-dimer has been shown to be useful. The clinical probability can be best assessed by wells score, which results in a classification of either DVT likely or DVT unlikely.
D-Dimer is a degradation product of cross-linked fibrin and therefore concentrations of D-dimer below a certain cut-off level are considered to indicate the absence of thrombosis(higher negative predictive value)
Wells Probability score for DVT <2 DVT unlikely, >2 DVT likely Clinical features points Active cancer 1 Paralysis,paresis,recent immobilisation of lower extremities 1 Localised tenderness along deep venous system 1 Entire leg swollen 1 Calf swelling>3cm larger than asymptomatic side 1 u/l pitting edema 1 Collateral superficial veins(non varicose) 1 Previously documented DVT 1 Alternative diagnosis as likely or more likely than DVT -2
NCCN Guidelines for Deep Vein Thrombosis Treatment in Cancer Patients
The therapy guidelines are aimed at in-hospital treatment and list the following drugs for anticoagulation:
*If patients cannot take anticoagulation medications, compression devices can be used, including graduated compression stockings
The guidelines suggest using low-molecular-weight heparin for long-term therapy for prevention of recurrent deep vein thrombosis in patients with advanced or spreading cancer. The minimum time for treating deep vein thrombosis is 3–6 months; pulmonary embolism requires 6–12 months of treatment.
Migrating venous thrombosis in acute leukemia
Musil D , Krc I .
Source: Department of Vascular Diseases, Institute of Pathological Physiology, Palacky University, Czech Republic.
Abstract: We present a 55-year-old man with acute migrating thrombophlebitis and deep vein thrombosis of muscle veins in both calves indicating occurrence of acute myelomonocytic leukemia. Thrombosis of superficial and deep veins of the lower limbs arose in spite of the adequate anticoagulation therapy with warfarin
Venous thromboembolism in patients with acute leukemia, lymphoma, and multiple myeloma
Ted Wun a , b , c, d, , and Richard H. White e
a Division of Hematology and Oncology, UC Davis School of Medicine, USA
The association of malignancies and venous thromboembolism (VTE) is a long held axiom in medicine. A growing number of studies have demonstrated that the risk of VTE associated with the hematological malignancies acute leukemia, lymphoma, and multiple myeloma is considerable. In fact, the incidence associated with these malignancies exceeds that for many solid tumors.
Review Article Thrombosis in children with acute lymphoblastic leukemia: Part III. Pathogenesis of thrombosis in children with acute lymphoblastic leukemia: effects of host environment
TE in association with childhood ALL is a multifactorial entity resulting from the interaction of the disease, chemotherapy and its effects, and possible prothrombotic states inherent to the host. The few studies conducted so far in children with ALL have reported wide variability in the prevalence of prothrombotic defects and its impact on the risk of TE.
Thrombosis Research Volume 111, Issue 6 , 2003, Pages 321-327
Venous thromboembolism in patients with acute leukemia: incidence, risk factors, and effect on survival
Journal of american society of hematology ( APRIL 23 2009)
Thombosis was a clinical presenting manifestation in 13 patients of 379 (3.4%, 95% CI 1.8%–5.8%): one with ALL (1.4%), three with APL (9.6%), and nine with non-M3 AML (3.2%). After exclusion of the four patients with arterial ischemic stroke (two with APL and two with non-M3 AML),the incidence of VTE at diagnosis was 3.2% in APL and 2.5%in non-M3 AML