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Showing posts with label HEAMATOLOGY. Show all posts
Showing posts with label HEAMATOLOGY. Show all posts

Mnemonic for Causes of pancytopenia

Cytopenia is a reduction in the number of each type of peripheral blood cell. A reduction in all 3 types of cellular components in peripheral blood is termed pancytopenia and this involves anaemia, neutropenia, and thrombocytopenia.

"All my blood has taken some poison"

  • Aplastic anemias
  • Megaloblastic anemias
  • Bone marrow infiltration
  • Hypersplenism
  • TB
  • SLE
  • Paroxysmal nocturnal hemoglobinuria

Initially, mild impairment in marrow function is inapparent and pancytopenia may become apparent only during times of stress or increased demand (e.g., bleeding or infection). More
severe degrees of cytopenias affect the peripheral blood count even in the steady state.

Severe pancytopenia is defined as follows :
i)Absolute neutrophil count < 500/mm3
ii)Platelet count < 20,000/mm3
iii) Corrected reticulocyte count < 1%
The peripheral smear shows pancytopenia with circulating blasts "High power"

iron deficiency anemia in pregnancy

A 22-year-old woman is pregnant and at 14-week gestation. Her hemoglobin level is 9 g/dL. She asks why she could have iron deficiency when she is no longer menstruating. Which of the following is the best explanation?
  • A.Occult gastrointestinal blood loss
  • B.Expanded blood volume and transport to the fetus
  • C.Hemolysis
  • D.Iron losses as a result of relative alkalosis of pregnancy

( B ). Iron deficiency occurs in pregnancy as a result of the expanded blood volume and active transport of iron to the fetus.

Anemias and it`s corresponding RBC`s shape

Erythrocytes may have abnormal shapes and sizes in certain diseases.In iron deficiency you would expect to see ?!
  • a.Microcytic, hypochromatic anemia with smaller mature erythrocytes
  • b.Macrocytic, hyperchromatic anemia with fewer, larger mature erythrocytes
  • c.Poikilocytosis and more fragile erythrocytes
  • d.Spherocytosis
  • e.No change in erythrocyte size or shape, but a substantial drop in the hematocrit

The answer is a.
Iron deficiency leads to anemia with the presence of smaller, pale-staining erythrocytes (microcytic, hypochromatic). Hyperchromatic, macrocytic anemia results from vitamin B 12 deficiency (answers b and e).

The presence of spherical rather than biconcave erythrocytes is known as spherocytosis (answer d). The RBC membrane undergoes deformation due to the inability of ankyrin to bind spectrin. The shape change results in trapping in the splenic sinusoids and excessive destruction of red blood cells in that organ. Poikilocytosis is the generic term for abnormally shaped erythrocytes (answer c).
Hereditary elliptocytosis and hereditary poikilo-cytosis are inherited diseases in which there is RBC membrane fragility and abnormal shape due to spectrin mutations. Mutations in Band 3 (anion exchanger 1) result in RBCs that are hyperchromatic with poikilocytosis.

Complications of Sickle cell disease

Medical mnemonic for the Sickle cell disease complications

SICKLE:

Strokes/ Swelling of hands and feet/ Spleen problems
Infections/ Infarctions
Crises (painful, sequestration, aplastic)/ Cholelithiasis/ Chest syndrome/ Chronic hemolysis/ Cardiac problems
Kidney disease
Liver disease/ Lung problems
 Erection (priapism)/ Eye problems (retinopathy)

Flow Cytometry


Student Documentary Film Assignment

Pelvic Spleen


A healthy, 23-year-old, nulliparous woman presented with lower abdominal pain, which she reported having had intermittently for the previous year. She had no other symptoms of gastrointestinal distress. A peripheral-blood smear showed no Howell–Jolly bodies.

Physical examination revealed a palpable mass in the suprapubic region. Abdominal ultrasonography revealed displacement of the spleen from its normal position and a homogeneous soft-tissue mass (measuring 11.0 by 9.3 by 4.2 cm) in the pelvis. Imaging of the liver and spleen after injection of technetium-99m sulfur colloid (Panel A) revealed a normal liver and a well-defined area showing abnormal accumulation of radiotracer (arrow) in the lower abdomen.
Multislice computed tomography, with three-dimensional reconstruction of a coronal image (Panel B), revealed the position of the spleen in the pelvis (black arrow), with torsion of the elongated pedicle (white arrow).

Wandering spleen, or pelvic spleen, is an uncommon condition associated with laxity or malformation of the suspensory ligaments of the spleen. Splenic torsion and infarction are potential complications. The principal therapeutic options are splenopexy and splenectomy. After discussion of treatment options, the patient declined surgical intervention and has continued to do so in follow-up over the past 3 years.

Pachyderma due to scabies

A 65-year-old white man with a history of multiple myeloma presented with thick, leathery, gray skin of the torso and extremities. Panel A shows the left axilla. The patient reported intense pruritus and thickening of his skin during the previous 6 months, despite treatment with oral and topical corticosteroids. Because of chronic immunosuppression due to his underlying malignant condition, a specimen obtained from scrapings of the skin was prepared and examined. It showed scabies mites, eggs, and scybala (fecal pellets) (Panel B), which confirmed a diagnosis of crusted scabies.

The patient was treated with oral ivermectin and topical permethrin, with noticeable improvement within 1 month; subsequently, the patient was lost to follow-up.
Crusted scabies is a rare variant of scabies and occurs most commonly in immunosuppressed patients.
It presents as: erythematous or gray hyperkeratotic patches or plaques that can resemble the hide of a pachyderm (elephant, rhinoceros, or hippopotamus); “pachyderma” refers to thick skin, like that of a pachyderm. Patients with crusted scabies have a very high burden of mites and are extremely infectious. Thus, a prompt diagnosis is needed to avoid transmission to others. No such transmission was documented in this case.

Megaloblastic anaemia is a disorder of the bone marrow. There is a presence of erythroblasts in the bone marrow with delayed nuclear maturation because of defective DNA synthesis.

In megaoblastic anaemia Erythrocytes are larger and have higher nuclear-to-cytoplasmic ratios compared to normoblastic cells.
Neutrophils can be hypersegmented, and megakaryocytes are abnormal. Risk Factors for Megaloblastic anaemia are Vitamin B12 deficiency ,Folic acid deficiency and Conditions with neither B12 nor folate deficiency, e.g. orotic aciduria, where there is a defect in pyrimidine synthesis, therapy with drugs interfering with DNA synthesis and myelodysplasia.

A White Hand Vs Red Hand

A 14-year-old girl presented for evaluation of 3 years of intermittent episodes of acute pain in the hands and feet. She had no other medical history. These episodes of pain were frequently triggered by physical exercise and bathing.

During physical examination, movement of the patient's right hand triggered burning pain, and the hand became acutely erythematous. Immersion of the right hand in warm water triggered the same kind of pain and erythema. The patient's presentation was typical of erythromelalgia, in which an increase in temperature triggers attacks of acute pain and red, swollen hands and feet. In some patients, pain and redness can extend beyond the distal extremities. Symptoms are relieved by exposure to cold.

Erythromelalgia can be seen in association with myeloproliferative disorders, connective-tissue disorders, vasculitis, diabetes, gout, multiple sclerosis, thrombotic thrombocytopenic purpura, and the use of certain drugs. Cases of erythromelalgia that are associated with myeloproliferative disorders usually respond to aspirin. Otherwise, treatment is difficult, although attendance at a pain rehabilitation center can be beneficial. The patient had no underlying disease, and no medical treatment was effective. She manages the erythromelalgia by avoiding triggering factors.

Hemostasis Lecture

A concise lecture on basics of hemostasis; the triggers of the development of the hemostatic plugs (primary) and (secondary). The differences between Hemostasis and Thrombosis.
Clinical presentation and importance are also discussed.

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