#paeds
see: [Dunn - leukaemia](x-devonthink-item://CDBBF2AC-B7F6-46A1-81B0-94C5B302F932), [Haeys' - acute leukaemia](x-devonthink-item://5230ED8C-F093-411D-812F-661EBC07E2AE)
*Leukemia* - Lymphoid or myeloid neoplasm with widespread involvement of bone marrow. tumour cells are usually found in peripheral blood
unregulated growth and differentiation fo WBC in bone marrow → marrow failure → anaemia, infections, and haemorrhage.
compare with *lymphoma* which has a discrete tumour mass arising from lymph nodes.
| type | findings | features |
| ---- | ------------------ | ---------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------- |
| ALL | - usually children | - T cell ALL can have a mediastinal mass (SVC-like syndrome)<br>- pancytopenia |
| CLL | - age > 60 | - mod splenomegaly<br>+/- lymphadenopathy<br>- high total WCC<br>- lymphocyte count > 20 <br>- pancytopenia late manifestation <br>- apheresis for very high WCC > 50, symptoms of vascular occlusion, etc |
| AML | - age ~ 65 years | - [[DIC]] is common presentation<br>- ↑ myeloblasts on peripheral smear<br>- splenomegaly<br>- pancytopenia <br>- |
| APML | - age > 65 | - very high D-dimer suggestive<br>- associated coagulopathy even if plt count mildly affected<br>- tx with all-trans retinoic acid |
| CML | age 45 - 85 | - philadelphia c/some<br>- dysregulated production of mature granulocytes (neutrophils, basophils)<br>- WCC > 100k<br>- anaemia common<br>- infections and bleeding rare<br>- *splenomegly always present* |
# Acute Leukaemias
> classic presentation is:
> 1. moderate-severe anaemia
> 2. thrombocytopenia
> 3. ↑ WCC
## ALL
- most common childhood type
- abrupt onset -- prominent [[Anaemia]] and **bruising**
- bone pain and secondary infection common
- [[lymphadenopathy]]
- hepatosplenomegaly -- can see on *ultrasound*!
- mediastinal adenopathy
- CNS involvement common
- pancytopenia
- circulating lymphoblasts
**Risks for ALL**
- family history
- immunosuppression
- alkylating agents (more AML than ALL)
- trisomy 21
- neurofibromatosis
- ataxia telangiectasia
- bloom syndrome
**Factors a/w good prognosis in ALL**
- age > 1 and < 10
- WCC < 50 x 10^9
- no testicular involvement
- no Trisomy 21
- no prior steroid exposure (risk of spont [[Tumour lysis syndrome]])
- no CNS disease
## AML
- usually age 70
- [[pancytopenia]]
- infection common
- bone pain
- splenomegaly
Risk factors:
- myelodysplasia
- radiation
- smoking
- benzene
- chemotherapy
types according to cytogenic markers M0 - M7
# Chronic leukaemias
> only seen in adults
> **splenomegaly is always present in CML**
- mild anaemia, pancytopenia common
- infections and bleeding are NOT common
## CML
- philadelphia chromasome → abnormal tyrosine kinase that causes cells to become malignant
# Complications
## Leukostasis
#radiology
- medical emergency in pts with leukemia, particularly AML and blast phase of CML
- over-accumulation of leukemic cells within the small vessels
- affects lungs and brain most commonly, presenting with symptoms of vascular oclusion
- often when WCC >100k
- consider in pt with myeloid leukemia wiht acute onset dyspnea and normal CXR or [[Pulmonary oedema]]