Haematological malignancies are clonal neoplasms that are derived from a single hematopoietic stem cell or peripheral lymphoid tissue.
Definition of terms
| Term | Definition |
|---|
| Blast cells | Immature precursor cells in the bone marrow |
| Myeloid lineage | Derived from bone marrow precursors |
| Lymphoid lineage | Derived from lymphocytes |
| Monoclonal proliferation | Expansion of a single clone of malignant cells. All cells are genetically identical. |
| Leukaemia | Malignant proliferation of bone marrow-derived haematopoietic cells with spillover into peripheral blood |
| Acute leukaemia | Rapid onset leukaemia with proliferation of immature cells (blasts) |
| Chronic leukaemia | Slow-onset leukemia with more mature cells |
| Lymphoma | Malignant proliferation of lymphocytes forming solid tumors |
| Myeloma | Malignancy of plasma cells |
| Remission | Remission is defined as less than 5% blasts in the bone marrow, normal PBF, and no other signs or symptoms of the disease |
- Risk factors
- Genetic factors
- Down syndrome has an increased frequency of Acute Leukemia.
- Bloom’s syndrome
- Fanconi’s anemia
- Wiskott Aldrich Syndrome
- Ataxia telangiectasia
- Klinefelter’s syndrome
- Radiation ****
- Infections
- EBV – Burkitt’s Lymphoma,
- HTLV-1 – Adult T-cell Leukemia and post-transplant lymphoproliferative disease
- HHV-8 – primary effusion lymphoma,
- HIV-1-associated high-grade B-cell lymphoma,
- Helicobacter pylori – MALToma,
- Malaria in Endemic Burkitt’s Lymphoma
- Chemicals
- Benzene – may cause bone marrow hypoplasia, dysplasia, Chromosomal abnormalities, myelodysplasia, and AML
- Drugs
- Alkylating agents (procarbazine and chlorambucil) – AML, especially when combined with radiotherapy
- Etoposide – an antileukemic that is associated with secondary leukemia
- Investigations
- Assessment of peripheral blood
- Complete blood count
- Peripheral blood film
- Bone marrow studies for cell morphology, histopathology, and immunohistochemistry
- Cytochemical studies with PAS, MPO, and Sudan Black
- Flow cytometry for Immunophenotyping. It is mainly used for lymphocytes to determine whether it is a B-cell or T-cell lineage.
- Cytogenetics and Karyotype Analysis
- Fluorescence in situ hybridization (FISH)
- Polymerase chain reaction (PCR)
- DNA Microarray
- Treatment
- Psychological support
- Nutritional support
- Pain management
- Transfusion and blood products
- Prevention of tumor lysis syndrome
- Antibiotics are indicated since neutropenia, hypogammaglobulinaemia, and impaired immune cell function may cause immunosuppression
- Intravenous fluid and maintenance of electrolyte balance
- Chemotherapy
- Remission induction
- Consolidation to destroy the remaining tumor cells
- Maintenance chemotherapy to maintain remission
- Radiotherapy
- Immunotherapy
- Stem cell transplantation
Biochemistry
| ACTH | P: <80 ng/L |
| ALT | P: 5–35 U/L |
| Albumin | P: 35–50 g/L |
| Aldosterone | P: 100–500 pmol/L |
| Alk. phosphatase | P: 30–130 U/L |
| α-Amylase | P: 0–180 IU/dL |
| α-Fetoprotein | S: <10 kU/L |
| Angiotensin II | P: 5–35 pmol/L |
| ADH | P: 0.9–4.6 pmol/L |
| AST | P: 5–35 U/L |
| Bicarbonate | P: 24–30 mmol/L |
| Bilirubin | P: 3–17 μmol/L |
| BNP | P: <50 ng/L |
| CRP | P: <10 mg/L |
| Calcitonin | P: <0.1 mcg/L |
| Calcium (ionized) | P: 1.0–1.25 mmol/L |
| Calcium (total) | P: 2.12–2.60 mmol/L |
| Chloride | P: 95–105 mmol/L |
| Cholesterol | P: <5.0 mmol/L |
| VLDL | P: 0.128–0.645 mmol/L |
| LDL | P: <2.0 mmol/L |
| HDL | P: 0.9–1.93 mmol/L |
| Cortisol AM | P: 450–700 nmol/L |
| Cortisol Midnight | P: 80–280 nmol/L |
| CK ♂ | P: 25–195 U/L |
| CK ♀ | P: 25–170 U/L |
| Creatinine | P: 70–100 μmol/L |
| Ferritin | P: 12–200 mcg/L |
| Folate | S: 2.1 mcg/L |
| FSH | P: 2–8 U/L ♂; >25 menopause |
| GGT ♂ | P: 11–51 U/L |
| GGT ♀ | P: 7–33 U/L |
| Glucose (fasting) | P: 3.5–5.5 mmol/L |
| Growth hormone | P: <20 mu/L |
| HbA1C (DCCT) | B: 4–6% |
| HbA1C (IFCC) | B: 20–42 mmol/mol |
| Iron ♂ | S: 14–31 μmol/L |
| Iron ♀ | S: 11–30 μmol/L |
| Lactate (venous) | P: 0.6–2.4 mmol/L |
| Lactate (arterial) | P: 0.6–1.8 mmol/L |
| LDH | P: 70–250 U/L |
| LH | P: 3–16 U/L |
| Magnesium | P: 0.75–1.05 mmol/L |
| Osmolality | P: 278–305 mosmol/kg |
| PTH | P: 0.8–8.5 pmol/L |
| Potassium | P: 3.5–5.3 mmol/L |
| Prolactin ♂ | P: <450 U/L |
| Prolactin ♀ | P: <600 U/L |
| PSA | P: 0–4 mcg/mL |
| Protein (total) | P: 60–80 g/L |
| Red cell folate | B: 0.36–1.44 μmol/L |
| Renin (erect) | P: 2.8–4.5 pmol/mL/h |
| Renin (recumbent) | P: 1.1–2.7 pmol/mL/h |
| Sodium | P: 135–145 mmol/L |
| TBG | P: 7–17 mg/L |
| TSH | P: 0.5–4.2 mU/L |
| T4 | P: 70–140 nmol/L |
| Free T4 | P: 9–22 pmol/L |
| TIBC | S: 54–75 μmol/L |
| Triglycerides | P: 0.50–2.3 mmol/L |
| T3 | P: 1.2–3.0 nmol/L |
| Troponin T | P: <0.1 mcg/L |
| Urate ♂ | P: 210–480 μmol/L |
| Urate ♀ | P: 150–390 μmol/L |
| Urea | P: 2.5–6.7 mmol/L |
| Vitamin B12 | S: 0.13–0.68 nmol/L |
| Vitamin D | S: 50 nmol/L |
Arterial Blood Gases
| pH | 7.35–7.45 |
| PaCO₂ | 4.7–6.0 kPa |
| PaO₂ | >10.6 kPa |
| Base excess | ±2 mmol/L |
Urine
| Cortisol (free) | <280 nmol/24h |
| Hydroxyindole acetic acid | 16–73 μmol/24h |
| Hydroxymethylmandelic acid | 16–48 μmol/24h |
| Metanephrines | 0.03–0.69 μmol/mmol cr. |
| Osmolality | 350–1000 mosmol/kg |
| 17-Oxogenic steroids ♂ | 28–30 μmol/24h |
| 17-Oxogenic steroids ♀ | 21–66 μmol/24h |
| 17-Oxosteroids ♂ | 17–76 μmol/24h |
| 17-Oxosteroids ♀ | 14–59 μmol/24h |
| Phosphate (inorganic) | 15–50 mmol/24h |
| Potassium | 14–120 mmol/24h |
| Protein | <150 mg/24h |
| Protein/creatinine ratio | <3 mg/mmol |
| Sodium | 100–250 mmol/24h |
Haematology
| WCC | 4.0–11.0 ×10⁹/L |
| RBC ♂ | 4.5–6.5 ×10¹²/L |
| RBC ♀ | 3.9–5.6 ×10¹²/L |
| Hb ♂ | 130–180 g/L |
| Hb ♀ | 115–160 g/L |
| PCV ♂ | 0.4–0.54 L/L |
| PCV ♀ | 0.37–0.47 L/L |
| MCV | 76–96 fL |
| MCH | 27–32 pg |
| MCHC | 300–360 g/L |
| RDW | 11.6–14.6% |
| Neutrophils | 2.0–7.5 ×10⁹/L (40–75%) |
| Lymphocytes | 1.0–4.5 ×10⁹/L (20–45%) |
| Eosinophils | 0.04–0.44 ×10⁹/L (1–6%) |
| Basophils | 0–0.10 ×10⁹/L (0–1%) |
| Monocytes | 0.2–0.8 ×10⁹/L (2–10%) |
| Platelets | 150–400 ×10⁹/L |
| Reticulocytes | 0.8–2.0% / 25–100 ×10⁹/L |
| Prothrombin time | 10–14 s |
| APTT | 35–45 s |
Paediatric
| Pulse Rate (bpm) |
|---|
| Neonate | 140–160 |
| Infant <1yr | 120–140 |
| 1–5 years | 110–130 |
| 5–12 years | 80–120 |
| >12 years | 70–100 |
| Respiratory Rate (tachypnoea) |
|---|
| 0–2 months | ≥60/min |
| 2–12 months | ≥50/min |
| 1–5 years | ≥40/min |
| >5 years | ≥30/min |
| Blood Pressure (mmHg) |
|---|
| Term | 65/45 |
| 1 year | 75/50 |
| 4 years | 85/60 |
| 8 years | 95/65 |
| 10 years | 100/70 |
| Weight Formulas |
|---|
| 3–12 months | (a + 9)/2 kg |
| 1–6 years | 2a + 8 kg |
| >6 years | (7a − 5)/2 kg |
| Haemoglobin (g/dL) |
|---|
| Term newborn | 13–20 |
| 1 month | 11–18 |
| 2 months | 10–15 |
| 1–2 years | 10–13 |
| >2 years | 11–14 |
| MUAC (6 months–5 years) |
|---|
| Obese | >17.5 cm |
| Normal | 13.5–17.4 cm |
| At risk | 12.5–13.4 cm |
| Moderate malnutrition | 11.5–12.4 cm |
| Severe malnutrition | <11.5 cm |
| Developmental Milestones |
|---|
| Social smile | 1.5 months |
| Head control | 4 months |
| Sits unsupported | 7 months |
| Crawls | 10 months |
| Stands unsupported | 10–12 months |
| Walks | 12–13 months |
| Talks | 18 months |
| CSF WBC (/mm³) |
|---|
| Term newborn | 0–25 |
| >2 weeks | 0–5 |
