Sickle Cell Disease (Sickle Cell Anemia): Complications

The following are the major diseases or complications that may be contributed to by sickle cell anemia (sickle cell disease):

Respiratory system (J00-J99)

  • Acute chest syndrome (ATS) – life-threatening condition; symptoms: Fever, cough, tachypnea (>20 breaths per minute at rest), chest pain (chest pain), leukocytosis (increase in the number of white blood cells in the blood), and pulmonary (“belonging to the lungs”) infiltrates
  • Pneumonia (inflammation of the lungs)
  • Pulmonary hypertension (pulmonary hypertension)

Eyes and eye appendages (H00-H59).

  • Orbital hemorrhages – (hemorrhages into the orbit).
  • Proliferative retinopathy – retinal disease associated with tissue overgrowth.

Blood, hematopoietic organs – immune system (D50-D90).

  • Bone marrow insufficiency – inability of the bone marrow to produce sufficient blood cells.

Skin and subcutaneous (L00-L99).

  • Ulus cruris – ulcer localized on the lower leg (usually in the lower third).

Cardiovascular system (I00-I99)

  • Hypoxic myocardial damage (myocardial damage caused by lack of oxygen).
  • Thrombosis

Infectious and parasitic diseases (A00-B99).

  • Bacterial infections up to sepsis (“blood poisoning”) – due to frequent splenic infarctions.

Liver, gallbladder, and bile ducts-pancreas (pancreas) (K70-K77; K80-K87).

  • Liver infarcts

Mouth, esophagus (food pipe), stomach and intestines (K00-K67; K90-K93).

  • Girdle syndrome (paralytic ileus (intestinal obstruction due to intestinal paralysis) caused by mesenteric infarction/obstruction of an intestinal vessel).
  • Ulcera duodeni (duodenal ulcers).

Musculoskeletal system and connective tissue (M00-M99).

  • Bone marrow necrosis → pain crises
  • Osteoporosis (bone loss)
  • Vertebral body cover plate collapses
  • Growth disorders

Ears – mastoid process (H60-H95)

  • Sensorineural hearing loss
  • Tinnitus (ringing in the ears)

Psyche – nervous system (F00-F99; G00-G99)

  • Enuresis (wetting)
  • Neuro-psychiatric changes – due to hemorrhage/infarction of the central nervous system.

Symptoms and abnormal clinical and laboratory findings not elsewhere classified (R00-R99).

  • Hematuria (visible blood in urine), painless – due to papillary necrosis.
  • Proteinuria – increased excretion of protein in the urine.
  • Vertigo (dizziness)

Genitourinary system (kidneys, urinary tract – sex organs) (N00-N99).

  • Hposthenuria (decreased concentrating power of the kidneys).
  • Renal dysfunction to renal insufficiency (kidney weakness).
  • Priapism – erection that lasts > 4 h without sexual stimulation; 95% of cases ischemic or low-flow priapism (LFP), which is very painful; LFP can lead to irreversible erectile dysfunction after only 4 h; therapy: blood aspiration and possibly intracavernosal (i.c.) sympathomimetic injection; “high-flow” priapism (HFP) does not require immediate measures.

Further

  • Organ infarcts, especially
    • Central nervous system (CNS)
    • Intestine
    • Lungs
    • Spleen
    • Kidney
    • Bone

Prognostic factors

An unfavorable outcome often occurs with the following factors:

  • Dactylitis – inflammation of fingers or toes.
  • Hemoglobin value < 7 g/dl
  • Leukocytosis – increase in the number of white blood cells.